EAP 2019 Congress and Master Course

Oral Presentations

735

Adolescent Medicine

Educational Attainment, Employment Status and Personal Autonomy of Young Adults with Neurodevelopmental Disorders

Zakhar Shchomak ¹ , Cláudia Lima ¹ , Sara Pereira ¹ , Manuela Baptista ¹

¹ Department of Pediatrics, Centro Hospitalar Universitário Lisboa Norte, Portugal

Background: Neurodevelopmental disorders are characterized by developmental deficits that produce impairments of personal, social, academic or occupational functioning.

Objective: To access the educational attainment, employment status and personal autonomy among adolescents aged 17-20 years followed in the neurodevelopment clinic of a tertiary hospital.

Methods: A telephonic survey was elaborated and conducted to assess educational level, employment status, professional internship enrolment, parental sensation of personal autonomy and other features like getting a bank account or a driving licence. Patients born in 1999 and 2000 followed in our centre from 2009 to 2018 were included. Clinical information comprising diagnosis, comorbidities and age at the first and the last appointments were retrieved from personal records.

Results: Fifty-two of 83 eligible patients (63%) responded to the survey; 69% were boys. The results were analysed by three major diagnosis groups i.e. Autism Spectrum Disorder (ASD) (n=10), Intellectual Disability (ID) (n=13) and Attention-Deficit/Hyperactivity Disorder (ADHD) (n=21). Concerning educational attainment, the majority were attending high school or higher levels of education (ASD 100%, ID 54% and ADHD 76%). Regarding employment the majority were enrolled in a professional internship or were employed (ASD 40%, ID 46% and ADHD 52%), while the rest were living at home under parental care or attending occupational centre activities. Parental sensation of personal autonomy was achieved in 60% of ASD, 54% of ID and 100% of ADHD patients. The number of adolescents obtaining a driving licence (ASD 40%, ID 15% and ADHD 85%) and owning/managing a bank account (ASD 40%/0%, ID 38%/15% and ADHD 90%/40%) were heterogenous among the groups.

Conclusion: Educational levels, employment possibilities and personal autonomy have been increasing among patients with neurodevelopmental disorders, however, vary significantly and depend on disorder itself and its severity. A better guidance is needed to support these adolescents in the transition to adulthood.

624

Adolescent Medicine

Characteristics of a Decade of Alcohol Intoxications in Adolescents in Pediatric Departments in Dutch Hospitals

Nicolaas van der Lelij ¹ , Loes de Veld ¹ , Joris van Hoof ²

¹ Pediatric department, Reinier de Graaf Gasthuis, Netherlands

² Behavioral Sciences Faculty, University of Twente, Netherlands

Aim: Admission of adolescents with an alcohol intoxication has become a health concern in current pediatrics. Over the years 2007 till 2016 we conducted a longitudinal study by monitoring the intake and treatment of 5,893 adolescents in all Dutch Pediatric Departments.

Methods: from 2007 till 2016 data were collected on all adolescents (aged younger than 18 and with a positive BAC), treated in all Pediatric Departments in The Netherlands. The Dutch Pediatric Surveillance System (NSCK) consists of a system in which all pediatricians report all admitted adolescents by completing a questionnaire, making use of a patient interview.

Results: 5,893 adolescents were treated in totally, most of them (4,678; 88%) related to severe alcohol intoxication; mean age was 15.4 years, and 48 % were girls. BAC level increased during this period (1.82 in 2007 and 2.01 in 2016), and reduced consciousness lasted from 2.24 hours in 2007 till 3.12 hours in 2016. Of the adolescents with alcohol intoxication, 11.4% had simultaneous drug usage. The attitude of the parents changed during the years: in 2011 (first year of registration) 68% of the parents gave permission to their child to drink alcohol, in 2016 this decreased to only 19%.

Conclusions: The last decades, alcohol intoxication among youngsters has become an important aspect in Pediatrics. The Dutch Surveillance System was used to conduct a longitudinal analysis on alcohol intoxication characteristics, medical treatment and effects of interventions in this group.

496

Allergy & Immunology

Assessment of Parent, Adolescent and Family Cognitive and Behavioral Dimensions Associated with Adherence to Treatment and Asthma Control

Luisa Barros ¹ , Margarida Custódio dos Santos ^1,2 , Ana Rita Oliveira ¹ , Raquel Nunes ¹ , Margarida Bonança ³ , Elsa Pereira ³ , Ana Margarida Neves ³

¹ Faculdade de Psicologia, Universidade de Lisboa, Portugal

² Psicologia, Escola Superior de Tecnologia da Saúde de Lisboa, Portugal

³ Consulta de Asmologia, Centro Hospitalar Universitário Lisboa Norte, Portugal

Background: Pediatric asthma management involves the development of a treatment plan and its implementation by the patient and the family. Adherence levels tend to be especially problematic during adolescence. Individual and family psychological dimensions contribute to a more comprehensive understanding of asthma management and control.

Objective: The aim of this study was to explore the relation between parents and adolescents with asthma, related knowledge and beliefs, family asthma management, and adherence to treatment and asthma control.

Methods: Sample of 112 adolescents with asthma, 11-18 years old and their caregiver filled measures of Asthma Knowledge and Beliefs about Asthma Medication. Physicians rated the asthma control and nurses rated adherence. A sub sample of 32 caregiver and adolescent dyads completed the Family Asthma Management System Scale (FAMSS).

Results: Show that both parents and adolescents have a reasonable knowledge about asthma, with wrong answers in similar domains, and overall positive beliefs regarding medication. Parents and adolescents knowledge about asthma were associated with asthma control, and with adherence for girls, but not for boys. Adherence was significantly associated with asthma control and with lower concerns about the secondary effects of medication. The overall family management and several dimensions of the FAMSS measure were positively associated with better adherence to treatment and asthma control.

Conclusion: FAMSS was a useful tool to identify domains of worse adaptation in families with lower adherence to treatment Understanding parents and adolescents asthma related knowledge and beliefs, and how the family organizes herself to manage the disease, may contribute to a more tailored educational intervention to increase adolescents levels of adherence and asthma control.

487

Allergy & Immunology

Visceral Leishmaniasis in a Patient with Hyper-IgM Syndrome Secondary to PIK3CD Mutation: A Case Report

Mariano Silva Hernández ¹ , Virginia Santana Rojo ¹ , Serafín Castellano Dámaso ¹ , María Bravo-García-Morato ² , Rebeca Rodríguez Pena ² , María Alba-Domínguez ² , Fernando Baquero Artigao ³ , Eduardo López-Granados ² , Francisco Javier Aracil Santos ³

¹ Department of Paediatrics, Hospital Universitario La Paz, Spain

² Department of Immunology, Hospital Universitario La Paz, Spain

³ Department of General Paediatrics and Paediatric Infectious Diseases, Hospital Universitario La Paz, Spain

Background: Visceral leishmaniasis is rare in patients with primary immunodeficiencies, especially, in patients with hypogammaglobulinemia. Recently, gain of function in PI3CKD have been related to a hyper-IgM syndrome, characterised by autoimmunity and lymphoproliferative disease, apart from hypogammaglobulinemia and predisposition to infections. As PI3CKD is part of the mTOR pathway, its treatment is based on blocking of different steps of it, with drugs such as sirolimus.

We present a case of visceral leishmaniasis in a 14-year-old boy with hyper-IgM syndrome secondary to PIK3CD mutation.

Case Presentation: Our patient is a 14-year-old boy with hyper-IgM syndrome secondary to PIK3CD mutation treated with immunoglobulin, sirolimus, cotrimoxazole, pantoprazole and calcium.

He had developed previous outbreaks of lymphoproliferative disease, consisting mainly of enlarged lymph nodes and tonsils, but they were controlled with sirolimus.

He referred a 6-week history of daily fever associated to headache and pain of his lymphadenopathies, more intense after the administration of immunoglobulin. On physical examination, he was pale and his lymphadenopathies were as usual, but slight splenomegaly was present. Laboratory tests showed a progressive pancytopenia (minimum values: haemoglobin 5 g/dL, WBC 1120/μL, neutrophils 610/μL, lymphocytes 290/μL, platelets 95000/μL). Sirolimus blood levels were higher than previous controls, without dosage changes nor renal disturbances. Sirolimus was discontinued, but there was not improvement of the symptoms and pancytopenia. Studies performed because of the pancytopenia were negative, except for positive PCR for Leishmania infantum in blood. PCR for Leishmania infantum was also positive in the bone marrow biopsy, although amastigotes were not visualized. Patient was treated with liposomal amphotericin B for 10 days, followed by additional doses at days 14 and 21. Progressively, fever, splenomegaly and analytic disturbances disappeared. After recovery, he received monthly suppressive therapy with liposomal amphotericin B for 6 months.

Conclusions: Visceral leishmaniasis should be considered as a possible cause of lasting fever in patients with immunodeficiencies, regardless of the type of immunodeficiency. Mainly, when fever is associated with pancytopenia and splenomegaly.

832

Critical / Emergency Care

Long-stay Patients in the Pediatric Intensive Care Unit

Joana Caldeira Santos ¹ , Francisca Martins ² , Marta Grilo ³ , Augusto Ribeiro ³

¹ Department of Pediatrics, Centro Hospitalar Vila Nova de Gaia/Espinho, Portugal

² Department of Pediatrics, Unidade Local de Saúde do Alto Minho, Portugal

³ Pediatric Intensive Care Unit, Centro Hospitalar de São João, Portugal

Background: Long-stay patients (LSP) in pediatric intensive care unit (PICU) are associated with high healthcare resource utilization.

Objective: To identify factors associated with long hospitalization in PICU.

Methods: Retrospective cohort study of all pediatric patients admitted in a PICU of Northern Portugal in 2017 and 2018. LSP were defined as patients having a length-of-stay greater than the 90^th percentile (≥ 14 days).

Results: A total of 626 patients were admitted, 10.7% (n= 67) were LSP, with a mean age of 7.2± 6.5 years and 59.7% (n=40) were male. Using univariate binary logistic models, we determine a significant association between admission from another intrahospital department (OR=0.35, 95% confidence interval [CI] 0.21-0.59; p0.001), emergency admission (OR=4.04, 95% CI 2.29-7.13; p0.001), nonsurgical admission (OR=2.67, 95% CI 1.59-4.45; p0.001), neurological disorder admitting diagnosis (OR=2.89, 95% CI 1.35-6.18; p=0.006), cardiovascular condition admitting diagnosis (OR=2.85, 95% CI 1.17-6.94; p=0.021), trauma related admission (OR=4.30, 95% CI 2.24-8.25; p0.001) and the length-of-stay. Additionally, it was tested the association between the previously independent variables and the length-of-stay in a multivariate regression model, retaining only the significant variables. An association was determined between nonsurgical and trauma admissions with longer length-of-stay, with corresponding OR_a of 4.68, 95% CI 2.54-8.63 and 9.04, 95% CI 4.23-19.33, respectively. The model classified correctly the observations in 89.3% of the cases. Model discrimination was assessed using a ROC curve, with corresponding AUROC =0.72 (95% CI=0.65-0.78; p0.001)

Conclusion: The clinical profile of LSP includes nonsurgical and trauma patients. A predictive model could help to identify patients with high risk of longer length-of-stay with a potential applicability for internal management regarding quality and cost-saving interventions.

669

Critical / Emergency Care

Acute Pain in Pediatric Emergency Settings: Challenges and Solutions

Kristina Ganzijeva ¹ , Ieva Kindereviciute ¹ , Lina Jankauskaite ^1,2 , Algirdas Dagys ^1,2

¹ Medical Academy, Lithuanian University of Health Sciences, Lithuania

² Department of Pediatrics, Hospital of Lithuanian University of Health Sciences Kauno klinikos, Lithuania

Introduction: Today, pain is characterized not only as physiological reaction and response to tissue damage, it is recognized as multidisciplinary issue and multi-profile problem. Over the last decades great progress was made in pediatric pain evaluation and pain management. Unfortunately, acute pediatric pain still remains misunderstood, under-diagnosed, and under-treated.

Aims: To investigate accuracy of acute pain assessment and management in Pediatric Emergency Department (PED) in Lithuania University of Health Sciences Hospital Kauno Klinikos (LSMU KK).

Methods: We performed a retrospective card record analysis before (year 2017) and after (year 2018) pediatric pain training course was conducted. In total, 1000 randomly selected outpatient card records were analyzed. All cases were divided into two groups: group A records from 2017, group B – from 2018. Cases were further divided into trauma and non-trauma and subdivided into 4 different age groups. We collected patient age, origin of pain, pain characteristics, pain score and medication.

Results: We compared 500 pain cases in each group. Group A and B consisted of 154 (30.8%) and 116 (23.2%) traumatic patients respectively. Pain was scored less in group A (420 children (84%)) comparing to group B (94.4% of all 500 cases, p0.001). In all age groups of group B pain was assessed more frequently and pain medication was prescribed more often compared to group A (p 0.001). There was a tendency to assess pain more often in non-traumatic patients in group A (p=0.054). However, pain relief in traumatic patients was less adequate compared to non-traumatic.

Conclusion: Pain evaluation differed in both groups. In group B pain was evaluated more frequently and received pain-medication more often than group A. Teenagers are still less likely to receive analgesics than toddlers. Tendency remains to give less painkillers to trauma patients compared to non-traumatic children.

512

Critical / Emergency Care

Task-Sharing is Associated with Reduced In-Hospital Paediatric Mortality in Sierra Leonean Hospitals

Christopher Hands ¹ , Sandra Hands ¹ , Emma Bailey ¹ , Mannah Janet ² , Mamasu Conteh ² , Dennis Marke ³ , Andrew Fryer ¹ , James Bunn ⁴

¹ Global Team, Royal College of Paediatrics and Child Health, UK

² Resus Department, Ola During Children's Hospital, Sierra Leone

³ Child Health Team, Ministry of Health and Sanitation, Sierra Leone

⁴ Child Health, World Health Organisation, Sierra Leone

Background: Following the Ebola epidemic in Sierra Leone, the Ministry of Health and Sanitation (MoHS) initiated measures to regain and extend improvements in healthcare for children. Within a wider strategy to reduce child mortality, UKAID supported MoHS and RCPCH to introduce locally adapted WHO Emergency Triage Assessment and Treatment (ETAT+) protocols to all district hospitals.

Objectives: Analyse operational programme data to identify trends in mortality rates and quality of care.

Methods: In 2017 two resident clinical mentors (one Sierra Leonean, one international) delivered a three-month on-the-job training programme alongside mentorship for nurses, with written and practical examinations (74% pass rate). Nurses passing the exams were authorised to initiate emergency paediatric treatment and were supported by clinical mentorship for three further months.

Mentors collected treatment data each day on children admitted the previous day and recorded discharge outcomes. These data were analysed to identify adherence to clinical protocols and to calculate mortality rates. In 2018, with intermittent support from RCPCH teams, admission and mortality data collected by hospital M&E officers were analysed in one-month blocks over six months.

Results: The 2017 data collection protocol was followed at 11 of 13 hospitals. Mean combined inpatient mortality over the first twelve weeks of the programme was 14%, and for the second twelve weeks was 9.6%. Adherence with clinical protocols improved for all emergency treatments, including appropriate use of oxygen (52% to 93%), correct antibiotic prescriptions (39% to 85%), and management of convulsions (54% to 83%). In 2018 data collection was undertaken at 12 of 13 hospitals. Combined mortality was 10.4%, and following further training fell to 7.3% in the second twelve weeks.

Conclusions: This analysis of operational data suggests that sustained reductions in hospital-level child mortality may be achievable in low-resource settings, when a task-sharing approach is combined with on-the-job training and mentorship.

188

Critical / Emergency Care

Faking it: Evaluating the Effectiveness of Low-Fidelity Simulation-Based Paediatric Teaching in a District Hospital

Michael Kershaw ¹ , Sobia Bilal ¹

¹ Paediatrics, Harrogate and District NHS Foundation Trust, UK

Background: Simulation-based teaching has become a key part of Paediatric training by allowing the development of the skills and knowledge to manage conditions and emergencies which are infrequently encountered in clinical practice. The current literature of simulation-based teaching focuses on large paediatric units with high-cost training programs, which could be prohibitively expensive for district hospitals.

Objectives: By conducting this prospective observational study we aimed to evaluate the low-fidelity ward-based simulation being conducted in Harrogate District Hospital. We aimed to demonstrate its effectiveness as well as assess participant’s attitudes towards this method of teaching.

Methods: Low-fidelity simulation-based teaching sessions were undertaken with a multi-disciplinary team during periods of low ward activity. They used a simple mannequin with expired, or spare, equipment and were run by the Consultant on the ward. Questionnaires were distributed to evaluate the participants attituded towards simulation as well as their confidence levels before and after simulation of Paediatric and Neonatal emergency scenarios. A total of 24 responses were received and included in this study.

Results: 100% of participants agreed, or strongly agreed, they wanted more simulation-based teaching. The average feedback from participants was agreement that these sessions improved their knowledge, communication, leadership, decision-making, and day-to-day practice. There was a statistically significant increase in participant`s confidence levels with regards to: preparedness to manage emergencies (p=0.001), understanding their role (p=0.008), leadership ability (p=0.002), escalating to seniors (p=0.014), practical skills (p=0.012), communicating effectively (p=0.025), and ability to recognise unwell children (p=0.034).

Conclusion: This study demonstrated that low-fidelity simulation undertaken at low cost is an effective and flexible teaching method which is regarded highly by participants. There is a high demand from trainees for more simulation-based teaching and we hope the flexibility and effectiveness demonstrated in this study helps to encourage more widespread use.

725

Critical / Emergency Care

Uncertain Result of Abdominal Ultrasound Produced by Undertrained Operator Increase the CT in the Pediatric Emergency Department

So Yeon Kim ¹ , Jae Yun Jung ¹ , Young Ho Kwak ¹ , Joong Wan Park ¹ , Hyun Jung Chung ¹ , Woo Ri Bae ¹

¹ Department of Emergency Medicine, Pediatric Emergency Seoul National University Hospital, South Korea

Background: Children with abdominal pain are commonly evaluated with ultrasound to reduce radiation exposure in the pediatric emergency department (PED). However, ultrasound is highly operator-dependent, and the results are varied according to the level of the operator. Uncertain result by the undertrained operator may increase the CT, paradoxically.

Objective: To compare the correlation between the level of ultrasound operator and the presence of an uncertain conclusion which leads to additional CT scans.

Methods: This study was a retrospective cohort pilot study conducted at a tertiary academic PED. Patients under 18 years old with abdomen ultrasound from January to March 2017 were eligible, and chronic disorders including malignancy, hepatobiliary disease, and previous abdominal surgery history were excluded. Patients were classified into ‘Specialist’ group (SG) operated ultrasound by radiology specialist, and ‘Resident’ group (RG) by radiology resident. In our institution, radiology specialist operated ultrasound during the working day (Monday to Friday, 9 AM to 6 PM, except holiday), and after working hours, radiology resident operated. We compared the rate of uncertain ultrasound conclusions and additional CT between two groups using Chi-square or Fisher’s exact test.

Results: A total of 106 were analyzed. Mean age was 4.84 years, and 68 (64.2%) were boys. SG were 62 (58.5%), and RG were 44 (41.5%). 22 (20.8%) patients had an uncertain conclusion. Among them, SG were 5 (22.7%), and RG were 17 (77.3%). (p 0.001). 7 patients underwent additional CT scans with an uncertain conclusion. Only 1(14.3%) patient in the SG and 6 (85.7%) in the RG. (p 0.001)

Conclusions: Abdominal ultrasound operated after working hours by radiology residents had a more uncertain conclusion and led to more CT imaging. If the radiology specialist was available at a time other than working hours, unnecessary CT scans might be reduced by about 70%.

833

Critical / Emergency Care

Syncope in the Young: Retrospective Review of Emergency Department Presentations

Leona Nertney ^1,2,4 , Jessica McGirr ² , Ciaran Finucane ³ , Roisin McNamara ¹ , Patrick Fitzpatrick ¹ , Paul McNally ^2,4

¹ Emergency Department, Children's University Hospital, Temple Street, Ireland

² Department of Paediatrics, Royal College of Surgeons in Ireland, Ireland

³ Medical Physics, St James's Hospital, Ireland

⁴ NCRC, National Children's Research Centre, Ireland

Background: Syncope is transient loss of consciousness due to global cerebral hypoperfusion, occurring in 1 in 5 children. 1-3% of visits to Paediatric Emergency services result from syncope or ancillary injury. Clinically challenging, acute unexplained episodes are compounded by lack of collateral history or fear of sinister pathology. Resulting over-investigation may occur, with increased burden on both patient and healthcare system.

Objective: This study sought to determine the current management practices of childhood syncope within a tertiary emergency department.

Methods: Retrospective review of presentations to TSCUH Emergency Department (January-December 2017). Symphony (Patient Information System was interrogated to extract cases with diagnostic codes: “Syncope”, “Presyncope”, “Vasovagal Episode” or “Faint” in 0-16-year olds. Information on demographics, presentation, event characteristics, clinical management and measures of healthcare utilisation were analysed. All data are reported as proportions, means+/-SD as appropriate and analysed in STATA14.0.

Results: Data from 104 patients diagnosed with syncope were analysed: mean age 11.5+/-3.4yrs; 73(70.2%) female. Family history was documented in 45(43.2%) cases. Normal physical examination in 101(97.1%); abnormalities noted in 3(2.9%) - cardiac murmur, bradycardia, hypotension. 66(63.5%) events were witnessed; 47(45.2%) were recurrent fainters. Event-related injuries were sustained in 26(25%). Key differentiating symptoms were undocumented in most cases. Events occurred largely in the standing position 57(54.8%), followed by situational syncope 14(13.5%). ECG was the most commonly performed investigation 86(82.7%), while more patients received urinalysis 29(27.9%) than lying/standing BP 20(19.2%). Referrals were initiated by parents in 73(70.2%) cases, 28(26.9%) were transported by ambulance. Average attendance duration was 3hrs33mins. Admission occurred in 4(3.9%) patients with additional 22(21.1%) referred for outpatient follow-up (highest referral rate to cardiology-50%).

Conclusion: Syncope is associated with varying levels of case complexity, resulting in high resource use. Adoption and implementation of International Clinical Guidelines which address these complex events should be considered, with proven positive impact on patient management.

197

Critical / Emergency Care

Children & Adolescence Injuries on the "New" Road with Electric Vehicles- COR (Children On Road) Study

Iris Noam Botton ¹ , Ayelet Shlez ¹ , Dania Takagi ¹ , Ehud Rosenbloom ¹

¹ Pediatric Emergency Department, Meir Medical Center, Israel

Background: Road accidents are a major cause of trauma. In recent years, there has been a significant change in the nature of accidents in children, with the increasing use of electric light vehicles.

Objective: To describe the characteristics of road accidents and injuries among children on "the new road" and to compare between electrical and non-electrical light vehicles accidents.

Method: Retrospective study, analyzing all referrals to pediatric ED for road accidents in children (

Results: 1531 children were included, of these, 205 were pedestrians, 602 were motor vehicle occupants, 504 were light vehicles riders, 203 were light-electric vehicle riders and 17 were motorcycle riders.

Distinct differences between groups were found in the average age, and severity of injuries. While only 2% of motor vehicle occupants were injured moderate/severe, in the other groups higher rates were found, between 17.3% (non- electric light vehicle), until 35.3% (motorcycle).

Light-electric vehicle riders, when compared to non-electric light vehicle riders, were older, more likely to have multiple systemic injuries (58.6% Vs. 31.8%), had higher Injury Severity Scores) 5.8 Vs 4.7), and more head injuries (18.7% vs. 13.1%), Chest injuries (3.4% vs. 1%) and external injuries (76.3% vs. 65.9%) (P0.05).

Conclusion: All of the unique characteristics of children and teenagers injured by electric bicycles: their age, the mechanism of the accidents, the characteristics and severity of the injuries, indicate that this is a new "player" that may be closer in nature to motorcycles and requires special attention.

There is room for increased awareness and education for safe riding, appropriate legislation and law enforcement. Further research is required in the field.

664

Critical / Emergency Care

To Improve the Quality of Paediatric Handovers by Introducing a New Safety Handover Tool and using Modified SBAR Based Handovers

Meenu Pandey ^1,2 , Imogen Norton ¹ , David Burnside ¹

¹ Paediatrics, Northampton General Hospital, UK

² Neonatology, Leicester Royal Infirmary, UK

Background: This Quality Improvement project was conducted with a view to improve the quality of morning handovers in a high turnover paediatric environment. The national recommendations suggest inclusion of a “sick” handover prior to the main handover to allow for a system risk assessment¹. Our aim was to create a new safety tool, and to introduce a new Modified SBAR (Situation, Background, Assessment, Recommendations) method to increase handover efficiency.

Objectives: The objectives were to increase the usage of new safety tool and Modified SBAR and in the morning handovers in the paediatric unit.

Methods: We conducted a pre-intervention questionnaire to assess the deficiencies in the existing SBAR based handovers. We then devised a new pre-handover safety tool called “SSSS” to discuss the number of Sick patients, Spacing, Staffing levels and Similar sounding names. Also, a new Modified SBAR handover method to include only relevant details of the patients was introduced. The senior trainees were trained in the use of the new handover tools. Two study cycles were conducted which involved modifications to the existing handover sheet. Post-intervention staff survey was done to see the results.

Results: The uptake of the SSSS tool was 85% and that of the Modified SBAR handover was 100% at the end of the study period. All the parameters to assess handover safety increased. Handover effectiveness increased from 81% to 95%.

Conclusions: We believe SSSS tool and modified SBAR based handovers are a new way to handover sick paediatric patients. This will also help to minimise errors and improve patient safety. We recommend their use in emergency and acute paediatrics settings for the future.

¹https://www.rcpch.ac.uk/sites/default/files/2018-03/facing_the_future_standards_for_acute_general_paediatric_services.pdf

550

Critical / Emergency Care

The Prevalence of Bacterial Infection in Recently Vaccinated Febrile Infants

Ali Raba ¹ , Ibraheem Krebit ¹

¹ Paediatric Emergency Department, Tallaght University Hospital, Ireland

Background and aims: Evidence is lacking regarding the best approach to evaluating recently vaccinated infants younger than 3 months who present to the emergency department (ED) with fever. There have not been any recent published studies assessing the prevalence of bacterial infections in this population. The aim of the present study is to investigate the prevalence of bacterial infections in young infants presenting with fever within 72 hours after vaccination.

Methods: We reviewed the electronic medical records of infants aged between 6-12 weeks who presented with a fever 38C to paediatric ED, Tallaght University Hospital, Dublin, from January 2018 to December 2018. Febrile infants who recently vaccinated within 72 hours prior to ED presentation were compared with those who did not recently vaccinated. Bacterial infection were diagnosed based on culture results (urine or blood or CSF).

Results: A total of 70 infants (age: 8.8±1.9 weeks, male: 40(57.1%)) were enrolled in this study. Among 70 infants, 19 (27.1%) had recently vaccinated, of whom 11(57.9%) presented to ED within 24 hours of vaccination, 7 (36.8%) presented 24-48 hours after vaccination and 1(5.3%) presented 48-72 hours after vaccination. The prevalence of bacterial infection in non-recently vaccinated infants was 17.6% compared to 10.5% in recently vaccinated infants. Interestingly, all vaccinated infants who had bacterial infection, were presented to ED with fever within 24 hours of vaccination and all bacterial infections in this group were urinary tract infection (UTI).

Conclusion: Fever should not be attributed only to the vaccinations in young Infants who present to paediatric ED with fever after recent vaccination. All febrile recently vaccinated infants should be carefully evaluated and at least urine testing should be done regardless of the time of vaccination after the presentation.

585

Critical / Emergency Care

Post-Enterovirus Polio-like Rhombencephalitis in a 6 Year-old

Andreas Trobisch ¹ , Raphael Ulreich ² , Miriam Pocivalnik ² , Klaus Pfurtscheller ² , Sebastian Tschauner ³ , Siegfried Rödl ²

¹ Neonatology, University Clinic for Paediatric- and Adolescent Medicine, Austria

² Division of Paediatric Intensive Care, University Clinic for Paediatric- and Adolescent Medicine, Austria

³ Division of Paediatric Radiology, University Clinic for Radiology, Austria

A 6-year old, intubated patient with intermittent CPR-necessity was admitted to the intensive care ward. He had fallen sick one week earlier with flu-like symptoms, which had progressed to head- and neck pain as well as vertigo. Furthermore he developed dyspnoea, hypersalivation, dysphagia and desaturations while failing non-invasive ventilation, leading to his intubation. Guillain-Barré-syndrome was assumed, IVIG given and extended with 3 days Methylprednisolone. Additionally 10 cycles of plasmapheresis were performed leading to a rapid improvement of recurrent reanimation necessary bradycardic-/asystolic events. Cerebral and spinal MRI showed signal alterations/oedema of the cervical anterior horn (2^nd motor neuron) from C2 to C5 as well as signal alterations of tegmentum pontis and medulla oblongata, suspicious for rhombencephalitis and anterior myelitis (Figure 1). At this point, the patient showed (sub)-total paralysis with only movement of a few hand and facial muscles. PCR screening for pathogens in tracheal fluid detected enterovirus and literature associates the MRI lesions with enterovirus D-68 infection. During weaning our patient demonstrated lack of diaphragmatic movement and thus effective breathing, which led to prolonged necessity of nasotracheal intubation and later on tracheostomy with (home)-ventilation. PEG tube was installed since he was unable to swallow effectively without risk of aspiration. Extensive rehabilitative therapies were initiated, leading to significant improvement of movement but remaining home-ventilation dependent. After 3 months on intensive care, he could be transferred to a rehabilitation clinic.

262

Critical / Emergency Care

Bulging Fontanelle in Febrile Infants as a Clinical Predictor of Bacterial Meningitis

Amit Ziv ¹ , Dania Takagi ² , Ayelet Shles ² , Ehud Rosenbloom ²

¹ Department of Pediatrics, Meir Medical Center, Israel

² Pediatric Emergency Unit, Department of Pediatrics, Meir Medical Center, Israel

Background: It is a common practice to perform a lumbar puncture in infants presenting with fever and a bulging fontanelle, in order to rule out bacterial meningitis; however, most of these infants have benign and self-limited diseases.

Methods: We performed a retrospective cohort study of febrile children with a bulging fontanelle who underwent lumbar puncture at the Meir Medical Center between the years 2005-2015. χ² test and descriptive statistics were used for data analysis.

Results: 764 children aged 2-18 months underwent a lumbar puncture in the Meir Pediatric Department during the study period. A total of 10 infants were diagnosed with bacterial meningitis. Only one infant (10%) presented with a bulging fontanelle.

304 patients had a bulging fontanelle and fever on their evaluation. Amongst them cerebrospinal fluid pleocytosis was found in 115 cases (37.8%), including one case of bacterial meningitis (0.3%). None of the infants described on admissions as appearing either well or moderate on presentation were found to have bacterial meningitis.

The leading diagnoses for bulging fontanelle in febrile infants in our study were upper respiratory tract infections, aseptic meningitis and acute otitis media.

Conclusions: The finding of a bulging fontanelle, particularly if it’s the only relevant sign in the infant’s examination, has a very low sensitivity and specificity for bacterial meningitis. Most of the causes for a bulging fontanelle in febrile infants are self-limited diseases. The routine approach of performing a lumbar puncture in febrile infants with a bulging fontanelle should be reconsidered.

567

Endocrine & Diabetes

The Effect of Associated Autoimmune Disease on Metabolic Control in Children and Adolescents with Type 1 Diabetes Mellitus

Simona Cainap ¹ , Claudia Bolba ¹ , Madalina Ursu ¹ , Adriana Bungardi ¹ , Georgia Tita ¹ , Bogdan Lucian ²

¹ Second Departament of Pediatrics, University of Medicine and Pharmacy "Iuliu Hatieganu", Romania

² Departament of Pediatrics, Emergency County Hospital "Dr Constantin Opris", Romania

Diabetes mellitus type 1(T1DM) is one of the most common chronic diseases in childhood, is caused by insulin deficiency resulting from the destruction of insulin-producing pancreatic beta cells.

Children and adolescents with T1DM are at increased risk for developing other autoimmune diseases (autoimmune thyroid disease, coeliac disease, Addison disease, AIJ, psoriasis, vitiligo, etc.). This could have an effect on the metabolic control of diabetes in the long term. The detection of concurrent autoimmune disease is important to prevent morbidity related to unrecognized disease.

Objective: The aim of this study is to assess the prevalence of different types of autoimmune disorders in a group of children with diabetes mellitus type 1 (T1DM) and to evaluate the effect of concurrent autoimmune condition (AI) on glycaemic control and growth.

Methods: A retrospective cross-sectional study was conducted on 152 patients with T1DM, aged between 1 and 18 years, who were followed up at the Second Pediatric Clinic Cluj-Napoca, from January 2011 to May 2016. The medical records and laboratory findings in the hospital’s electronic system of all patients were reviewed.

Results: The prevalence of associated autoimmunity was 26.97% in our population. Out of the 41 patients, 27 (17.6%) had autoimmune thyroid disease, 9 (5,92%) had coeliac disease, and 3(1,97) had juvenile arthritis, 1(0,65%) had psoriasis and 1(0,65%) had vitiligo. Addison’s disease was not diagnosed in this cohort of patients. There was no significant difference either in growth parameters or HbA1c between children with associated autoimmune disease and T1DM compared with children with T1DM.

Conclusions: The prevalence of concurrent autoimmune disease is greater in children with T1DM than in general population. The effect of having another concomitant autoimmune condition on metabolic control of diabetes and growth is not clear.

327

Gastroenterology & Nutrition

Is Endoscopy Useful in the Investigation of Chronic Abdominal Pain in Children?

David Croaker ¹ , Himasha Nanayakkara ²

¹ Department of Paediatric Surgery, The Canberra Hospital, Australia

² Medical School, University of Notre Dame, Sydney, Australia

Background: Investigation for chronic or recurrent abdominal pain is frequently requested in paediatric surgery. The senior author had felt that endoscopy in this setting is rarely useful. Accordingly a retrospective review of our unit’s practice was performed to ask the question "how often is endoscopy useful in managing children with chronic abdominal pain?

Objective: To review the usefulness of gastroscopy in the investigation of chronic paediatric abdominal pain.

Methods: Retrospective chart review of all paediatric gastroscopies performed during the last 15 years. This paper focuses on those referred for abdominal pain. Demographic data, reason for referral, and pathological findings were reviewed and compared with children referred for other reasons.

Results: 141 patients referred for investigation of non-specific abdominal pain were reviewed, from a total of 1779 paediatric endoscopies. 75 were female, and 66 male. The average age of those referred for pain was 11.02 years. The average age of 1638 referred for all other reasons was 7.89 years. (p 0.01)

41% of children had completely negative examinations. 30% had a gastropathy, and 29% microscopic findings of oesophagitis, contrasting with 30% of children gastroscoped for all indications who had a gastropathy. Only those being investigated for coeliac disease were more likely to have reactive gastropathy: 54%.

Specific diagnoses included eosinophilic oesophagitis(EoE): 5; Helicobacter gastritis: 3; other acute gastritis: 5; coeliac disease: 5; gastric or duodenal polyps: 2.

Conclusion: 141 scopes were performed to make few specific new diagnoses. Low grade chronic changes are commonly reported in both stomach and oesophagus regardless of presentation, and are positively correlated with coeliac disease. The diagnostic yield is not sufficiently high to recommend indiscriminate use of endoscopy in non specific abdominal pain. It is not clear that mucosal changes always explain symptoms of chronic abdominal pain in children.

242

Gastroenterology & Nutrition

The Impact of Oral Nutritional Supplementation on Body Mass Index in Children Treated for Cancer

Ugur Demirsoy ¹ , Feza Kirbiyik ² , Ali Evrim Dogan ² , Funda Corapcioglu ¹

¹ Pediatric Oncology, Kocaeli University, Faculty of Medicine, Turkey

² Advanced Medical Nutrition, Nutricia, Turkey

Objectives and the Study: This was a retrospective single center study where records from malnourished pediatric cancer patients were collected and analyzed from Kocaeli University Hospital, Pediatric Oncology, Turkey. The aim was to assess the impact of oral nutritional supplementation on body mass index (BMI) in malnourished children treated for cancer.

Methods: Inclusion criteria included a diagnosis of cancer and all standard treatments completed in the department according to the standard protocol regimens for the specific indications. Patients were considered malnourished if weight for height was below -2 SD as determined from gender specific WHO growth charts. The cohorts included patients treated during 2010-2016 time period. Patients who received oral nutritional supplementation (ONS) in addition to their specific anti-cancer regimen were recorded for their nutritional status based on weight, height and BMI during their follow up visits. Records were obtained for a period of 8 months from the start of the treatment.

Results: A total of 60 pediatric cancer patients with malnutrition (%64,6 male, %35,4 female) were included in the study. Data were analyzed either for all patients (total) or after clustering in to following tumor groups; CNS tumors 13.3 % (8 patients), lymphoma 18.3% (11 patients), other tumors 68.3% (41 patients). Comparison of pre-ONS and post-ONS anthropometric data has shown an increase in bodymass index (BMI) for the total patients group data with an increase in BMI for 36 patients (60%) versus decrease in BMI in 24 patients (40%) (p 0.05) (Figure 1). Statistically significant increase was also observed for other tumors group (24 patients versus 17 patients) (p 0.001) and for lymphoma group (8 patients versus 3 patients) (p=0.012). Although not statistically significant, there was an increase in BMI for CNS tumors group as well (4 patients versus 4 patients (p=0.068).

Conclusions: ONS is an important intervention in pediatric cancer patients that increases BMI and improves the nutritional status. The extent of improvement is possibly related to the disease type and location

576

Gastroenterology & Nutrition

Undernutrition in Two Different Communities of School-Children in North of Mozambique

Sofia Simões Ferreira ¹ , Joana Caldeira Santos ¹ , Andreia Teles ¹ , Mulaja Étienne ¹ , Carla Rêgo ¹

¹ Department of Pediatrics, Vila Nova de Gaia/Espinho Hospital Center, Portugal

In Africa, the number of stunted children has steadily increased, reaching 58.7 million in 2017, Mozambique showing one of the highest prevalence’s. Within the same country there may be nutritional differences depending on the proximity to the cities.

Characterize children from two communities of Mozambique, regarding sociodemographic indicators as well risk factors for malnutrition.

Longitudinal and interventional study in school-children from two communities in northern Mozambique [Ilocone (rural community) and Namicopo (city community)]. Children from 6 months to 12 years were invited to participate during their school-time. Demographic and anthropometric data (weight, brachial perimeter, length) were evaluated. WHO criteria were used to characterize nutritional status. Nutritional education and referral to primary care were provided.

77 children were evaluated: 35 from Namicopo, mean age of 68 months (8-131 months), 65,7% females; 42 from Ilocone, mean age of 40 months (6-96 months), 59,5% females. Significant differences (p0.05) were observed between the communities: in Ilocone the children were younger; births occurred more frequently at home; higher consumption of animal protein, beans and fruit; less consumption of grains; father with higher unemployment and age and poorest households. Prevalence of undernutrition was: 74.3% in Namicopo [with greater prevalence of mild wasting (54,3%)] and 52.4% in Ilocone [mainly stunting (31%)]. Wasting was significantly (p0,05) associated with less consumption of beans and fruit, prolonged duration of breastfeeding and single-parent families whereas stunting to poorest households, father`s unemployment, progenitor´s lower level of education and breast milk for shorter periods.

Undernutrition was diagnosed in more than half of this young population, the rural and the urban community showing a higher prevalence of chronic and acute undernutrition respectively. In our sample, poorest households were more significantly associated with stunting and poor feeding with wasting. Implications on present and future health of this population are of concern.

201

Gastroenterology & Nutrition

Maternal Antimicrobial Use at Delivery Affects Gut Microbiota of Infants into the Early Weaning Period

Naruaki Imoto ¹ , Fumitaka Amanuma ² , Hidekazu Maruyama ³ , Shin Watanabe ¹ , Naoyuki Hashiguchi ⁴

¹ Department of Microbiome Research, Juntendo University, Japan

² Neonatology, Iwate Prefectural Iwai Hospital, Japan

³ Pediatrics, Iwate Prefectural Iwai Hospital, Japan

⁴ Department of Emergency and Disaster Medicine, Juntendo University, Japan

Background: Intestinal colonization by bacteria such as Bifidobacterium or Bacteroides in infants and their presence in the gut at specific time points are known to affect the health of the host later in life. There are few published studies on the impact of maternal antimicrobial use at delivery on the gut microbiota in infants during the first year of life. To investigate the effect of antimicrobial administration immediately before delivery as intrapartum antibiotic prophylaxis (IAP) on the enteric environment in infancy around the weaning period and one year old.

Methods: A cross sectional study was conducted on healthy Japanese infants aged 6 months (n=23) and 12 months (n=26) to investigate the effect of antibiotic administration immediately before delivery as intrapartum antibiotic prophylaxis (IAP) on the enteric environment in infancy by using next generation sequencing to analyse the diversity and the composition of gut microbiota.

Results: In total, the fecal samples of 23 6-month-old and 26 12-month-old infants were analysed by next generation sequencing. Two 6-month-old infants and one 12-month- old infant were excluded because they had received antimicrobial drugs between birth and the sampling day. Antimicrobial agents were given systemically to the mothers at delivery for cesarean section, Group B Streptococcus-positive status, and premature rupture of membrane cases. IAP at the time of delivery does not influence the overall microbial diversity in the gut microbiota but decreased the occupancy by Bacteroides significantly (p=0.03), even in a subgroup analysis in infants group who were born by vaginal delivery at 6 months (p=0.02). In contrast, no influence of IAP was observed on gut microbiota in 12 months-old infants group.

Conclusion: Antimicrobial use immediately before the delivery are associated with decreased occupancy of Bacteroides in infants around weaning period. Although the current study provides a significant understanding of the gut microbiota in infancy, a follow-up study for a longer term is needed to identify the clinical significance of the change in the composition of gut microbiota based on the results.

251

Gastroenterology & Nutrition

A Pilot Study of Randomized Controlled Trial of Bowel Preparation for Pediatric Colonoscopy

Pornthep Tanpowpong ¹ , Hansa Sriphongphankul ¹ , Chatmanee Lertudomphonwanit ¹ , Suporn Treepongkaruna ¹

¹ Pediatrics, Faculty of Medicine Ramathibodi Hospital, Thailand

Background: Adequate bowel preparation is crucial for colonoscopy though no standardized regimen exists for children. Polyethylene glycol with electrolyte solution (PEG-ELS) is a widely used osmotic agent due to its good efficacy and high safety profile. However, some children may not tolerate large volume and poor palatability. Previous meta-analyses in adults comparing between a full single dose vs. split doses of PEG-ELS showed that the split dose group had a higher rate of successful bowel preparation. Similar study in children is limited.

Objectives: We compared the efficacy (i.e., successful bowel preparation) of using PEG-ELS as a full single dose vs. split doses for bowel preparation before pediatric colonoscopy. The secondary outcomes were tolerability, acceptability, and compliance between two regimens.

Methods: Investigator-blinded randomized controlled trial was conducted to enroll children aged 2-18 years who underwent an elective colonoscopy at a teaching hospital between March 2018 and February 2019. Patients were randomly assigned to receive PEG-ELS as a full single dose or two split doses. The Boston Bowel Preparation Scale was used for the efficacy (i.e., successful if score ≥ 6). Tolerability, acceptability, and compliance were evaluated by using a standardized questionnaire.

Results: A total of 43 colonoscopies (21 in the full single dose and 22 in the split dose group) were performed. Median age was 9.1 years old (IQR: 5.2-13.6). We found a higher rate of successful bowel preparation in the split dose group (95% vs. 71%, P= 0.046). Significant lower rate of nausea/vomiting (36% vs. 71%, P= 0.02) and higher rate of willingness to repeat the protocol (82% vs. 33%, P= 0.001) in the split dose group were also noted.

Conclusion: The split dose regimen of PEG-ELS for bowel preparation before pediatric colonoscopy provides a superior efficacy, potential tolerability and acceptability as compared to the traditional full single dose regimen.

427

Gastroenterology & Nutrition

Meta-Analysis of 196 Articles: Plasma Phospholipid Fatty Acid Supply of Expecting Women during Pregnancy and at Delivery

Eva Szabo¹, Tamas Marosvolgyi², Laszlo Poto², Tamas Decsi³

¹ Department of Biochemistry and Medical Chemistry, University of Pecs, Hungary

² Institute of Bioanalysis, University of Pecs, Hungary

³ Department of Paediatrics, University of Pecs, Hungary

Background: As long-chain polyunsaturated fatty acids play an important role in the maturation of the developing nervous system, maternal fatty acid supply is essential for the fetus as well as the newborn baby.

Objective: Our aim was to systematically review available data on fatty acid composition of plasma and erythrocyte membrane lipids in expecting women during pregnancy and at delivery.

Methods: Electronic literature search was performed in July 2017, on Embase, Cochrane Library, and Ovid Medline with a search strategy including the following search expressions: (pregnant* OR gestation* OR deliver*) AND (arachidonic OR docosahexaenoic) NOT animal. We analysed data from clinical trials investigating fatty acid composition of plasma or erythrocyte membrane lipids in healthy expecting women without any fatty acid supplementation during pregnancy. Statistical analysis of fatty acid data was performed by Comprehensive Meta-Analysis v3.0 Software.

Results: After excluding duplicates there were 3351 potentially relevant articles. We excluded 3155 articles because they didn’t meet our inclusion criteria, so 196 relevant articles remained publishing fatty acid data of plasma or erythrocyte membrane lipids, while 39 articles published data of plasma phospholipids (PLs). During the first trimester the studies investigated fatty acid status of a great number of mothers (n = 8337-8555), while in the three other timepoints the studies investigated somewhat smaller (n = 802-2757) populations. Values of arachidonic acid decreased significantly by the 3^rd trimester, while its values became significantly higher at delivery. On the other hand, values of docosahexaenoic acid remained quite stable during pregnancy, but decreased by delivery (Table).

Conclusion: 1. Our results indicate that on a population level, docosahexaenoic acid status remains remarkable stable during pregnancy 2. However, in plasma phospholipids values of arachidonic acid decreased significantly in the course of pregnancy.

774

Genetic & Metabolic

Comparing Cognitive Functions of Children with PKU and Controls using CANTAB Nonverbal Tests

Dora Becsei ¹ , Ildiko Erni ¹ , Erika Kiss ¹ , Erika Simonova ¹ , Attila Szabo ^1,2 , Ildiko Szatmari ¹ , Petra Zsidegh ¹ , Janos Bokay ¹

¹ 1st Department of Paediatrics, Semmelweis University, Hungary

² Pediatric and Nephrology Research Group, Hungarian Academy of Sciences, Hungary

Background: Phenylketonuria is an inherited error of metabolism caused by the failure of the phenylalanine hydroxylase enzyme. In poorly-treated cases, the patients have mental retardation, psychiatric problems and skin diseases.

Objective: We aimed to examine the cognitive functions of children with PKU comparing them with age-related control patients. We examined the correlation between cognitive functions and serum Phe, Tyrosine, Phe/Tyr ratio.

Methods: The children were examined with 5 nonverbal tests of Cambridge Neuropsychological Test Automated Battery including tests of working memory, learning and executive function; visual, verbal and episodic memory; attention, information processing and reaction time.

The data were analyzed using Microsoft Excel and R statistical packages. We used Mann-Whitney tests to compare the two groups. We normalized the results of each task and obtained a 0-10 score system to characterize the mean cognitive functions. The correlations were analysed with Spearman probes.

Results: We collected 54 children with PKU and 99 controls. We got the most explicit difference in SOC subsequent thinking time: the children with PKU were 3.12 times slower (1668 ms vs. 533,4 ms). The results show a correlation between cognitive functions with age and Phe. The mean normalized cognitive function was 5,77 in the group of children with PKU, and 7,52 in the control group.

Conclusion: Some CANTAB tests showed a significant difference between PKU and control patients – the most significant one: SOC subsequent thinking time.

According to our results, the nonverbal computer tests can be used very well to examine the fine cognitive dysfunctions caused by PKU, and it has a correlation with the serum Phe. The results confirm the importance of a strict diet.

223

Genetic & Metabolic

Metabolic Syndrome and its Components among Egyptian Obese Adolescents’ Girls: Influence of ApolipoproteinE Polymorphism

Nayera Elmorsi Hassan ¹ , Abeer Atef El Ashmawi ² , Sahar Abd-El-Rauf El-Masry ¹ , Waheeba Ahmed Zarouk ³ , Marwa Farouk Mira ² , Gamila SM El-Saeed ⁴ , Omar Hosni Dwidar ¹

¹ Biological Anthropology Department, National Research Centre, Egypt

² Pediatrics Department, Cairo University, Egypt

³ Molecular Genetics and Enzymology Department, National Research Centre, Egypt

⁴ Medical Biochemistry Department, National Research Centre, Egypt

Metabolic complications with obesity are increasing in childhood and extend to adulthood.

Aim: To assess the prevalence of metabolic syndrome (MS) among a sample of Egyptian adolescent girls and investigate its association with Apolipoprotein E.

Subjects & Methods: A cross sectional study included 200 Egyptian adolescent girls aged between 12-18 years.They were subjected to lood pressure measurement, anthropometric measurements (weight, height and waist circumference), Laboratory investigations (fasting glucose and lipid profile) and molecular analysis (Apo E).

Results: Overweight/obese girls were suffering significantly more than normal weight girls from hypertension (66.7% vs40.8%),diabetic (46.7% vs 31.2%) and low HDL (64% vs 59.2%).Girls withMS had significant higher values of BMI-Z score, WC, blood pressure, cholesterol and triglycerides, and significant lower HDL. Allele E3 was more frequent among girls with MS (59.1% vs 55.1%), while allele E4 was more frequent among girls without MS (41% vs 36.4).Genotype E3/E4 was more frequent among girls with MS (45.5% vs 23.1%), while genotype E3/E3 was more frequent among girls without MS (43.6% vs 36.4%). MS was the most prominent among E3/E4 genotype (35.7%) which had the highest frequency of elevated cholesterol, triglycerides, LDL and blood glucose, while genotype E2/E4; which was rare among both groups; had the highest frequency of elevated blood pressure (68.8%) and low HDL (71.4%).

Conclusion: MS was significantly more prominent among overweight/obese girls compared to the normal weight ones, and among E3/E4 genotype; which had the highest frequency of disturbed lipid profile and blood glucose.

776

Genetic & Metabolic

Biochemical Comparation Between Infants with Deficiency of Vitamin B12 Secondary to Maternal Deficiency and due to Genetic Disorder

Sara Teixeira ¹ , Teresa Campos ¹ , Esmeralda Rodrigues ¹ , Teresa Cardoso ² , Hugo Rocha ³ , Laura Vilarinho ³ , Elisa Leão-Teles ¹

¹ Centro de Referência de Doenças Hereditárias do Metabolismo, Centro Hospitalar de São João, EPE, Portugal

² Serviço de Medicina Interna, Centro Hospitalar de São João, EPE, Portugal

³ Unidade de Rastreio Neonatal, Metabolismo e Genética, Instituto Nacional de Saúde Doutor Ricardo Jorge, Portugal

Background: Vitamin B12 or Cobalamin (Cbl) plays an important role in many metabolic pathways. Its deficiency can lead to a wide spectrum of hematologic and neurological disorders that can be reversed or minimized by early diagnosis and prompt treatment. Cbl deficiency in newborns/infants may be secondary to nutritional deficits or due to defects in the absorption, transport or intracellular metabolism of Cbl, and can be identified in Portuguese newborn screening by analysis of acylcarnitine profile.

Objective: Compare biochemical parameters of infants with deficiency of Cbl secondary to maternal deficiency to infants with genetic deficit.

Methods: Included infants referred due to suspicion of Cbl deficit, between 2009 and 2018. Data from neonatal metabolic screening and infants and mothers clinical files.

Results: Were identified 8 breastfed infants, 4 with deficiency secondary to maternal cause and 4 with CblC disorder (an inborn error of the intracellular metabolism of Cbl, secondary to mutations in MMACHC gene). Seven were referred in the first month of life, due to alterations in neonatal screening, and one was diagnosed at 5 months of age when evaluated by growth and development impairment.

In newborn screening, the mean levels of propyonilcarnitine and propyonilcarnitine/acetylcarnitine ratio was two times higher in infants with genetic disorder. In these patients the serum Cbl was higher (10 times), as well as, the levels of methylmalonic acid (3.5 times) and homocysteine (4 times). Contrariwise, the mean levels of methionine were 1.6 times higher in nutritional deficit.

Conclusion: The mean values of the acylcarnitines, methylmalonic acid, and homocysteine are higher in the genetic CblC deficiency, when compared to nutritional deficits. However, the size of this sample does not allow conclusions regarding of cutoffs for each parameter.

476

Infectious Diseases

Prognostic Factors in Late-Onset Streptococcus agalactiae Sepsis and Bacteremia

Serafín Castellano Dámaso ¹ , Raquel Gómez García ² , José David Andrade Guerrero ¹ , Inés De Augusto Claudino ⁷ , Virginia Santana Rojo ¹ , Mariano Silva Hernández ¹ , Marta Dorado Criado ¹ , Celia Fabra Garrido ¹ , María Fátima Ara Montojo ¹ , Rosario López López ³ , María de Ceano-Vivas la Calle ³ , Julia Martín Sánchez ³ , María Pilar Romero Gómez ⁴ , Iker Falces Romero ⁴ , Juan José Menéndez Suso ⁵ , Cristina Verdú Sánchez ⁵ , Miguel Sáenz de Pipaón Marcos ⁶ , Laura Sánchez García ⁶ , Luis Escosa García ¹ , Cristina Ots Ruiz ¹ , Fernando Baquero Artigao ¹ , Ana Méndez Echevarría ¹ , Teresa del Rosal Rabes ¹ , Luis Alfonso Alonso García ¹ , Francisco Javier Aracil Santos ¹

¹ General Paediatrics and Infectious and Tropical Diseases Department, Hospital Universitario La Paz, Spain

² Centro de Salud Alpedrete, Atención Primaria Madrid, Spain

³ Emergency Department, Hospital Universitario La Paz, Spain

⁴ Microbiology Department, Hospital Universitario La Paz, Spain

⁵ Pediatric Intensive Care Unit, Hospital Universitario La Paz, Spain

⁶ Neonatalogy Department, Hospital Universitario La Paz, Spain ⁷ Pediatrics Department, Centro Hospitalar Oeste, Portugal

Background: Streptococcus agalactiae or Group B Streptococcus (GBS) is one of the main causes of sepsis in newborns and young infants. GBS is also a major cause of meningitis in newborns, and it might lead to neurological impairment in those children affected. Disease manifestations are often non-specific, and symptoms are of short duration before diagnosis is made.

Objective: To determine possible prognostic factors of late-onset Group B streptococcal sepsis, diagnosed at a single tertiary care hospital in Madrid, Spain.

Methods: Retrospective review of culture-proven GBS sepsis, after 7th day of life, between 2000 and 2013.

Results: Sixty-eight episodes of late-onset GBS sepsis or bacteremia were diagnosed. Clinical onset started during after-birth hospitalization in 10 % of patients. GBS was identified in blood culture (67 cases) and/or CSF (14 cases). Sixteen (24%) presented meningitis. Mean age at diagnosis was 34 days (8-125 days). Mother’s rectal-vaginal culture was positive for GBS in only 14.5%, negative in 58% and was not performed in 27.5%. The most common clinical symptoms were: fever (73%), irritability (58%) and food rejection (52%). Three patients died (4.4%). Seven patients developed severe neurological impairment.

Adverse outcome (death or neurological impairment) in the univariate analysis was related to lower temperature, lower WBC count, lower platelets, lower neutrophils, higher immature/total neutrophil ratio (I/T) and male gender (p0.05). With logistic regression analysis, variables related to adverse outcome with p 0,1.

Conclusion: Despite preventive measures, GBS remains a leading cause of sepsis and meningitis in newborns. GBS continues to cause mortality and frequent neurological sequelae. GBS sepsis seems to be more severe in male patients, in those with lower fever, lower WBC, lower neutrophil and lower platelet count and in those with higher immature neutrophil count.

502

Infectious Diseases

Is Vertical Transmission of Chlamydia trachomatis a Health Problem in Spain?

Marta Dorado Criado ¹ , Celia Fabra Garrido ¹ , Elena Merino San Martín ² , Carolina González Arboleya ² , Patricia González Donapetry ³ , Bartolomé Gómez Arroyo ³ , Fernando Baquero Artigao ⁴ , María De la Calle Fernández-Miranda ² , María Inmaculada Quiles Melero ³ , Cristina Calvo Rey ⁴

¹ Pediatrics, Hospital Universitario La Paz, Spain ² Ginecology, Hospital Universitario La Paz, Spain ³ Microbiology, Hospital Universitario La Paz, Spain ⁴ Pediatric Infectious Diseases, Hospital Universitario La Paz, Spain

Background: C. trachomatis infection is the most prevalent sexually transmitted disease (STD) in Europe. Maternal infection increases the risk of premature rupture of membranes and low birthweight and could be transmitted to the newborn through the birth canal, eventually causing nasopharyngitis, conjunctivitis and pneumonia.

Objective: The main objectives were to analyse the prevalence of C.trachomatis infection in young women and the rate of transmission to the newborn. Secondary objective was to evaluate the need for screening programs for C. trachomatis in this population.

Methods: We conducted a prospective and interventional pilot study of 71 pregnant women aged 15-25 years. Polymerase chain reaction (PCR multiplex BDmax) for STD (Chlamydia, Neisseria gonorrhoeae, Trichomonas) was performed in a urine sample during the postpartum-period.

Newborns from C. trachomatis infected mothers were tested for Chlamydia using PCR in nasopharyngeal aspirate and urine samples. Mothers were interrogated about Chlamydia symptoms in the newborns.

Results: Median age was 23 years (IQR 17-23) and 70% were immigrants, most of them (60%) from Central and South America. The prevalence of Chlamydia infection was 19.7% (14 cases, 70% from immigrant mothers). We also detected 2 N. gonorrhoeae and 3 Trichomonas infections. Two infected mothers presented premature rupture of membranes (at 36 weeks gestation) but no newborn had low birthweight. Two children born to infected women (14%) were positive for Chlamydia. One infant had positive PCR in both urine and nasopharyngeal aspirate, and developed conjunctivitis. The other one had a positive PCR in urine and developed no symptoms. Both mothers and infected infants were treated with azithromycin.

Conclusions: We have found a high prevalence of Chlamydia infection in young pregnant woman, with a 14% rate of perinatal transmission. Our results support the need for screening programs in this target population.

373

Infectious Diseases

The Greatest Impact on Parents Opinion about Children Vaccination is made by Health Care Specialists

Agne Navickaite ¹ , Vitalija Mesceriakova ¹ , Paulius Kalibatas ² , Ginreta Valinciute ³ , Stefano del Torso ^5,9 , Diego van Esso ⁶ , Zachi Grossman ⁷ , Adamos Hadjipanayis ^8,9 , Arunas Valiulis ^2,3,4,9 , Algirdas Valiulis ¹⁰

¹ Medical Faculty, Vilnius University, Lithuania

² Medical Faculty Institute of Clinical Medicine, Vilnius University, Lithuania

³ Medical Faculty Institute of Health Sciences, Vilnius University, Lithuania

⁴ Clinic of Children‘s Diseases, Vilnius City Clinical Hospital, Lithuania

⁵ Pediatra di Famiglia, Pediatra di Famiglia, Italy

⁶ Primary Care Service Muntanya, Catalan Institute of Health, Spain

⁷ Maccabi Health Services, Maccabi Health Services, Israel

⁸ Department of Paediatrics, Larnaca General Hospital, Cyprus

⁹ European Academy of Paediatrics, European Academy of Paediatrics, Belgium

¹⁰ Medical Faculty, Institute of Clinical Medicine, Department of Rehabilitation, Physical and Sports Medicine, Vilnius University, Lithuania

Background: Global vaccination is one of the greatest achievements in medicine, which resulted in the decrease of life-threatening illnesses. However, over the past decade, the number of people who doubt the benefits of vaccination has increased.

Objective: To evaluate the opinion about vaccines of parents raising pre-school children and how different sources of information shape that opinion.

Methods: A questionnaire survey was conducted in Vilnius kindergartens in the period from December 2017 to May 2018. 329 parents of children aged from 1 to 4 participated in the survey. The respondents were compared by gender, education level and primary care provider (paediatrician vs family doctor). The data are processed by SPSS 22.0 program, the difference between the compared groups is considered significant when p≤0.05.

Results: Parents who at least once have visited homeopath value vaccines benefit 6.33 out of 10 points. However, parents who did not apply to homeopaths tend to evaluate the benefits of the vaccine more favorably (8.25 out of 10 points, p≤0.05).

Majority of respondents have received negative information about vaccination. 229 parents at least once have found negative information on the internet, but tend to rate benefits of vaccines quite well: 8.24 out of 10 points. Furthermore, 17.3 perc. respondents, who at least once received negative information about vaccination from health care specialists, tend to evaluate vaccinations worse: 6.40 out of 10 points. More than 20 perc. of parents are reported at least one episode of negative information about vaccines received during visite of health care specialist.

Conclusions: The greatest impact on parents` vaccines related negativism is done by healthcare specialists, but not internet or other mass media. Future research is needed due to necessity of deeper analysis of primary sources and ways of spreading of vaccines related information in the families of preschool children.

264

Infectious Diseases

Prevalence of Hepatitis B Infection in Children of Eastern Nile (Khartoum State)

Amin Alagib Mohamed Musa ¹ , Altayeb Abdulmonem ² , Homeida Mamoun ¹ , A.S.Ali Shymaa ¹ , A.A.Ibnouf Sondos ¹

¹ Paediatrics, University of Medical Sciences and Technology, Sudan

² Department of Surgery, Ibn Sina Hospital Ministry of Health, Khartoum, Sudan

Background: The global prevalence of Hepatitis B is important with2 billion people exposed. The younger the person infected the more serious are the outcomes in term of complications. The World Health Organization estimated that 100 million persons have chronic hepatitis B in Africa.

Screening for Hepatitis B was introduced in Sudan in 2002 as part of the extended program of Immunization. In Sudan, Hepatitis B positivity ranged from 6.8% to 26.0%.

Methods: A screening for hepatitis B positivity was carried on a sample of 894 school -children (839 from Darfur and 55 from North State). The variables collected were age, area of residence, history of exposure to Hepatitis B, family past and parent history of exposure to risk factors classified as low, average and high. The data collected were summarized in term of frequency tables to estimate prevalence and chi-square tests were used to determine association between variables.

All statistical tests were considered significant when p 0.05.

Results: The overall prevalence was 4.1% (37/894). Across the age groups the prevalence was higher (4.7%, 30/639) in those aged 10-14 years with no statistically significant difference between age group (p=0.381). No positive case was recorded in outside Darfur (0/55) whereas in Darfur the positivity was 4.6% (37/802). No statistically significant association was found between Hepatitis B positivity with past history (p=0.196), family (p=0.117) and parent’s (p=0.539) history and level of exposure to risk (p=0.09).

Conclusions: Our findings revealed a prevalence of 4.1% which was out of the ranged of the national prevalence of 6.5%-26.0%; this difference may be due to the impact of the extended program of immunization.

331

Infectious Diseases

Bacteriophage Therapy in Pediatrics: Pros & Cons

Karaman Pagava ¹

¹ Child & Adolescent Medicine, Tbilisi State Medical University, Georgia

Introduction: The emergence of antimicrobial resistance and increase in side effects of antibiotic therapy has become a critical problem in modern medicine. As a rule the new antibiotics are more toxic, corresponding damages can have cumulative nature. The above-mentioned points to the necessity of optimization of the stewardship of antibiotic therapy, especially in children. Bacteriophage therapy is considered as an effective tool to improve the treatment of bacterial infections and minimalize its adverse consequences. Pediatricians’ knowledge in this area is not sufficient.

The goal of the study was to conduct a survey of the literature regarding bacteriophage therapy (BT), define its Pros and Cons.

Materials: The published literature of last twenty years was analyzed. Our own experience, exceeding more than 30 years was taken into consideration as well.

Results: Pros – the bacteriophage therapy does not cause toxicity, allergy, dysbacteriosis, it is safe and can be used even in premature babies and pregnant women, combined with all kind of medicines, it has no contraindications.

It is claimed that bacteriophages can be used effectively instead or with the antibiotics in the treatment of different bacterial infections (sepsis, gastroenteritis, dermatitis, osteomyelitis, pneumonia, urinary tract infections) caused by following agents - Proteus, Klebsiella, Escherichia, Shigella, Pseudomonas, Salmonella, Streptococcus, Staphylococcus.

Bacteriophages may have some immunomodulation abilities too.

Cons - The paucity of appropriately conducted placebo-controlled clinical trials,

Regulatory conundrum of BT.

BT (at least when bacteriophages are given PO) can induce production of anti-bacteriophage antibodies, which might decrease the efficacy of BT; In children, particularly in infants their production is less manifested.

Conclusions: BT is efficient and safe, but further investigations are needed.

173

Infectious Diseases

Coronary Arterial Lesions and Intravenous Immunoglobulin Resistance in Kawasaki Disease: A Case-Control Study

Virginia Santana Rojo ¹ , Mariano Silva Hernández ¹ , Pablo Fernández Fraga ⁷ , Marta Dorado Criado ¹ , Celia Fabra Garrido ¹ , Serafín Castellano Dámaso ¹ , José David Andrade Guerrero ¹ , Marta Bautista Barea ¹ , Ursino Adrián Barrios Machain ¹ , Lucía Deiros Bronte ³ , Ángela Uceda Galiano ³ , Antonio J. Cartón Sánchez ³ , Carlos Labrandero de Lera ³ , Luis García-Guereta Silva ³ , José Antonio Ruiz Domínguez ⁴ , Marta Bueno Barriocanal ⁴ , Miguel Ángel Molina Gutiérrez ⁴ , Monserrat Bret Zurita ⁵ , Ana Gómez Zamora ⁶ , Miguel del Río García ⁶ , Sara Murias Loza ⁷ , Luis Alfonso Alonso García ² , Luis Escosa García ² , Fernando Baquero Artigao ² , Talía Saínz Costa ² , Cristina Ots Ruiz ² , Cristina Calvo Rey ² , Ana Méndez Echevarría ² , Teresa del Rosal Rabes ² , Francisco Javier Aracil Santos ²

¹ Department of Paediatrics, Hospital Universitario La Paz, Spain

² Department of General Paediatrics and Paediatric Infectious Diseases, Hospital Universitario La Paz, Spain ³ Department of Paediatric Cardiology, Hospital Universitario La Paz, Spain

⁴ Paediatric Emergency Department, Hospital Universitario La Paz, Spain

⁵ Department of Paediatric Radiology, Hospital Universitario La Paz, Spain

⁶ Paediatric Intensive Care Unit, Hospital Universitario La Paz, Spain

⁷ Department of Paediatric Reumatology, Hospital Universitario La Paz, Spain

Background: The most important complication related to Kawasaki Disease (KD) are coronary arterial lesions, mainly aneurisms. Although several drugs have been used for this disease, the only treatment that decreases coronary lesions is intravenous immunoglobulin (IVIG). However, fever persists or reappears in 20% of the patients with KD treated with IVIG, which is called IGIV resistance. IGIV resistance has been linked to an increased risk of coronary damage.

Objective: The aim of this study was to compare the frequency and the severity of coronary arterial lesions among resistant and responsive KD patients treated with IVIG in a Spanish tertiary hospital.

Methods: We performed a case-control study. It included 38 KD patients who were resistant to IVIG (cases) in a period of 16 years. We chose 2 IVIG responsive KD patients (controls) per each case, the immediately previous and after ones.

Results: The incidence of resistance to IVIG in our KD patients was 18%.

Coronary damage was present in 50% of our resistant patients, whilst its incidence in responsive patients was 13%. Furthermore, resistant patients suffered severe coronary arterial lesions more frequently compared to responsive patients (11% vs 1%). Not only severe lesions were more usual, but also the mild ones (21% vs 7%).

The extension of injury was higher in resistant patients. 24% of the unresponsive patients showed 3 or more vessels affected, while none of the responsive patients had such extensive damage.

Moreover, all the coronary arterial lesions were transient in patients with good response, but in 16% of the unresponsive patients, lesions were permanent.

Conclusion: Resistance to IVIG is quite common in our KD patients. It is related to higher risk, severity, extension and duration of coronary damage. Thus, prediction, early detection and close follow-up are necessary to adequate the treatment and improve the prognosis of these patients.

850

Infectious Diseases

EBV or ZVZ… Which one to blame? A case of ADEM

Mariana Simoes ¹ , Gonçalo Vale ¹ , Maria São Pedro ¹ , Rita Matos Parreira ¹ , Susana Correia ¹ , Elisabete Gonçalves ¹

¹ Pediatrics, Centro Hospitalar Barreiro-Montijo, Portugal

Background: Acute Disseminated Encephalomyielitis (ADEM) is an immune-mediated demyelinating disorder which can occur following viral infections or vaccination and spontaneously. Several viruses can be implicated, including Epstein-Barr Virus (EBV) and Varicella Zoster Virus (VZV). Fever, headache, and meningeal irritation signs are the most common initial findings followed by seizures, focal neurological deficits and altered consciousness. Diagnosis is clinical and radiologic, being Magnetic Ressonance Imaging (MRI) the most important imaging exam. With appropriate treatment, prognosis is generally good.

Objective: To alert to less common etiologies and manifestations of ADEM

Methods: Report a clinical case of a child admitted to a suburban hospital in Portugal.

Clinical Report: 4-year old boy, admitted in the Pediatric Emergency Room (ER) with depression of consciousness and conjugate eye deviation to the right. In the previous 10 days, the family reported headache, frequent vomiting and fluctuant prostration, without fever. He also had recent history of chickenpox (20 days before admission). Blood analysis were unrevealing. Examination of cerebrospinal fluid (CSF) revealed pleocitosis (54 cells/mm3) with slight predominance of polymorphonuclear cells. He was hospitalized for investigation and started intravenous acyclovir. Cranioencephalic (CE) Computerized Tomography (CT) showed no alterations. Electroencephalogram documented diffuse slow-wave activity and CE-MRI revealed thalamic and subcortical lesions suggestive of ADEM. CSF microbiological culture was negative and multiplex Polimerase Chain Reaction for neurotropic virus was positive for EBV. Acyclovir was suspended at the 8^th day of hospitalization. He remained asymptomatic since the 6^th day after admission and was discharged after stopping acyclovir. He was posteriorly readmitted for immunoglobulin administration.

Conclusion: ADEM can be difficult to diagnose. Frequently, the etiologic agent remains unknown. In this case, EBV might be considered as an opportunistic agent in the setting of a transient immunosuppression state. We also highlight EBV as an important causative agent of ADEM.

651

Neonatology

Effect of High-Resolution Computed Tomograhy (HRCT) Results in Prognostic Evaluation of Bronchopulmonary Dysplasia (BPD) Cases

Abdullah Baris Akcan ¹ , Seyhan Erisir Oygucu ² , Ahmet Gokhan Arslan ³ , Deniz Ozel ⁴ , Nihal Oygur ⁵

¹ Department of Pediatrics, Division of Neonatology, School of Medicine, Aydin Adnan Menderes University, Turkey

² Department of Pediatrics, The Kyrenia University, Dr Suat Günsel Hospital, Cyprus

³ Department of Radiology, Akdeniz University, Turkey

⁴ Department of Biostatistics and Medical Informatics, Akdeniz University, Turkey

⁵ Department of Pediatrics, Division of Neonatology, School of Medicine, Akdeniz University, Turkey

Objectives: BPD carries a risk of long term pulmonary sequale with changes in pulmonary mechanics and parenchyma. HRCT is a method of detecting such structural changes. Our aim was to characterize these structural abnormalities associated with BPD and to evaluate whether the severity of HRCT changes was associated with clinical findings.

Methods: 28 patients born with a mean gestation age of 30 ± 2.9 weeks and diagnosed as BPD in their neonatal period were reevaluated when they were between the post natal ages of 6-12 months. They were divided into three groups as mild (n:8), moderate (n: 10), and severe (n: 10) according to their clinical data. HRCT scans were performed in patients with a history of moderate and severe BPD. One radiologist analysed scans using a structured scoring system.

Results: Their ages were between 9.8 ± 2.3 months at time of reevaluation.The average HRCT total score of patients was respectively moderate 7.20 ± 4.05 ; 7.40 ± 2.84 with severe BPD.The differerence between them was not significant (p = 0.620).Data obtained during the neonatal period, there was no significant correlation between the duration of oxygenation, the duration of mechanical ventilation, birth weights and the total HRCT scores performed at the time of reevaluation, in patients with moderate and severe BPD (p = 0.408, r = 0.183 for birth weights).

Conclusions: Those data suggest that the pathology detected in radiological imaging may not always be parallel to clinical findings and that the clinical course of infants with severe radiological data may be better or worse than expected. However, we believe more studies with larger number of patients and with different scoring systems are necessary in order to evaluate the role of HRCT in the clinical evaluation and long term prognosis of BPD.

139

Neonatology

Acute Reduction in Neonatal Brain Injury by Selective Blockage of CB1 and Activation of CB2 Receptors

Daniel Alonso-Alconada ¹ , Silvia Carloni ² , Andrea Duranti ² , Ainara Pintor-Rial ¹ , Antonia Alvarez ¹ , Enrique Hilario ¹ , Walter Balduini ²

¹ Department of Cell Biology and Histology, University of the Basque Country, Spain

² Department of Biomolecular Sciences, University of Urbino “Carlo Bo”, Italy

Background: Cannabinoids (CBs) can modulate a wide range of neurological and physiological processes through the activation of their receptors, CB1 and CB2. CB receptor agonists and antagonists have demonstrated to produce either protective or toxic responses after brain ischemia. URB447, a CB1 antagonist and CB2 agonist, can selectively block CB1 and activate CB2 receptor signaling.

Objective: Using an in vivo model of neonatal brain injury, to examine the potential therapeutic effect of URB447, given before or after a hypoxic-ischemic (HI) event.

Methods: 7-day-old rat pups received a single intraperitoneal injection of URB447, i) 1 hour before starting the ischemic procedure (HI+URB447 1h-PRE), ii) 30 minutes (HI+URB447 30min-POST) or iii) 3 hours (HI+URB447 3h-POST) after hypoxia. To test if the protective effect of URB447 was mediated by CB1/CB2 modulation, WIN-55212, a CB1/CB2 receptor agonist, was injected i.p. 1h and 30 minutes before HI (HI+WIN-55212) or 30 minutes before URB447 administration (HI+URB447+WIN-55212). Each HI-injured group (HI) received a corresponding volume of vehicle. N=10 for all groups. The neuroprotective effect of drugs was assessed using histological and molecular techniques in 14 day-old animals.

Results: HI-induced reduction in whole hemisphere (46.5%) and cerebral cortex (65.7%) areas was avoided after URB447 administration, showing a decrease in brain infarction both before (whole hemisphere: 83.7%; cerebral cortex: 92.7%) and 30 min after (whole hemisphere: 95.7%; cerebral cortex: 95.4%) the injury (Fig.1). WIN-55212 completely blocked the neuroprotective effect of URB447 (Fig.2). Of clinical relevance, URB447 injected 3 hours after HI reduced brain infarction 95.7% and 88% in whole hemisphere and cerebral cortex, respectively (Fig.1). Furthermore, delayed cell death, astrogliosis and white matter damage returned to those values observed in sham animals.

Conclusion: Selective blockage of CB1 and activation of CB2 receptors induce an acute reduction in brain injury in the neonatal rat. Grants: UPV/EHU (GAITUZ2019, GIU17/018).

810

Neonatology

Inhaled Nitric Oxide for Pulmonary Hypertension and Bronchopulmonary Dysplasia in Extreme Prematurity; Benefits with Targeted Use

Deepa Hariharan ¹ , Ganesh Veluswami ¹ , Velmurugan Kannappan ¹ , Pangaluru Sarathkala ¹

¹ Neonatology, Sooriya Hospital, India

Background: Preterm infants are at risk for early pulmonary hypertension (PH) that is associated with high risk of bronchopulmonary dysplasia (BPD) or death. While inhaled nitric oxide (iNO), is an effective treatment for PH in term infants, there is insufficient evidence for BPD prevention; however, the preterm infants were not screened for early PH and iNO was not targeted for PH in these studies. We hypothesized that early detection of PH and targeted iNO therapy will improve respiratory outcomes in extreme prematurity.

Aim/ methods: 164 infants less than 30 weeks gestation who needed ventilation during the 18 month study period were enrolled. On echocardiographic (ECHO) diagnosis by peaditaric cardiologist, 46 infants were detected to have PH at 72 - 96 hours of age. They were randomized to receive iNO at 5ppm (study group, n=23) or placebo (control group, n=23) till 14 days of age or resolution of PH, whichever was earlier. ECHO was done every 48 hours in both groups. The demographic profile, ventilator and surfactant use, PDA closure, nutrition and inotrope use were similar in the 2 groups. The 2 groups were analyzed for oxygenation index (OI) at 7 days, incidence of BPD and death.

Results: The mean OI on day 7 in the study and control groups were 9.7 and 14.1 respectively (p0.05). Duration of ventilation in the study and control groups were 13.6 days and 19.6 days respectively (p0.05). 4 infants in the study group, and 8 in the control group had BPD (p0.05). There was 1 death in each group.

Conclusion: Early detection of PH and targeted iNO therapy improves oxygenation and reduces BPD in extreme prematurity.

455

Neonatology

Exploring Nurses’ Perspective about Helping Babies Survive Programme in Western India – Qualitative Findings

Naveen Thacker ¹ , Robert Clark ² , William Keenan ³ , Deep Thacker ¹ , Mayur Shinde ⁴ , Richard Bell ⁵ , Kandarp Talati ⁶ , Somashekhar Nimbalkar ⁴

¹ Pediatrics, Deep Children Hospital, India

² Pediatrics, Brigham Young University, USA

³ Pediatrics, Saint Louis University, USA

⁴ Pediatrics, Pramukhswami Medical College, India

⁵ Pediatrics, NorthBay Medical Center, USA

⁶ Department of Interdisciplinary Research, Foundation for Diffusion of Innovations, India

Background: Helping Babies Survive (HBS) program was piloted in five districts of India during 2016-17. Landscape analysis evaluated changes between HBS and non-HBS healthcare facilities to understand how the program had influenced newborn care practitioners and practices.

Objective: To understand nurses’ perception towards usefulness of HBS trainings and experiential feedback for further strengthening HBS implementation and scale-up

Methods: Interview guideline was developed and perception explored through in-depth interviews. Total 14 nurses were interviewed (9 HBS trained, 5 untrained). Manual content analysis was done to report the findings under pre-defined thematic domains - Delivery and Newborn Care Practices; and HBS Skill Station/Practice Sessions/Continuous Quality Improvement(CQI).

Results: Nurses perceived that HBS training, CQI initiatives, visits by mentors and program officers(PO) helped in skill improvement. It helped improve birth preparedness, patient communication during labor, delivery per abdomen, skin to skin care(SSC) and early initiation of breastfeeding. HBS training also improved self-confidence to administer initial care, including resuscitation, to babies not crying/unstable who were earlier referred out without any interventions. Nurses learnt that not all babies require bag & mask or ventilation, and many a times baby responds to rubbing or stimulation.

Nurses were able to practice at designated HBS skill stations within their facilities, however organizing peer practice sessions were challenging. Shortage of staff, shift duty, staff attrition/rotation and workload were cited as primary reasons for inability to regularly practice. Visits by HBS POs were catalytic in facilitating peer practice sessions, discussing real life challenges and having shared insights and hence are of paramount importance to ensure CQI.

Conclusion: Nrses perceived that HBS training and mentoring visits helped deliver essential newborn care even at remote facilities. Further implementation and scale-up of HBS program should emphasize facility visits by HBS POs/mentors on monthly basis and strategies to address known facility-level barriers to CQI.

556

Neonatology

Introducing a Lactation Consultant into a NICU Service Improves Early Access to Breast Milk

Margaret Broom ^1,2 , Judith Smith ¹ , Mary Ellen Youseman ¹ , Alison Kent ^3,4

¹ Dept of Neonatology, Centenary Hospital for Women and Children, Canberra Hospital, Australia

² University of Canberra and ACT Health, SYNERGY: Nursing and Midwifery Research Centre, Australia

³ Division of Neonatology, University of Rochester, Golisano Children's Hospital at URMC, USA

⁴ Medical School, Australian National University, Australia

Background: Providing breast milk for extremely premature and sick neonates has documented benefits. Lactation consultants (LC) have become important additions to neonatal intensive care unit teams to improve provision of maternal breast milk, particularly in the early hours after birth.

Aim: To assess the impact of the introduction of a permanent LC on: 1) provision of breast milk for neonates; and 2) education and lactation support for mothers and staff.

Methods: A mixed method study including a retrospective chart audit and a pre and post LC staff survey. Information collected included: first expressed breast milk, episodes of lactation education and support. Survey questions provided staff feedback regarding: education access to support and meeting maternal needs. Data was analysed using descriptive statistics and Chi-square tests.

Results: 161 charts were included (82 pre LC and 79 post LC). Post LC service there was a significant increase in maternal access to LC appointments (12/82 vs 63/79; p 0.01) as well as breast pump education (53/82 vs 64/79; p0.01). There was an increase in EBM provision at 12 hours postnatally (38/82 vs 48/79; p0.01). Staff surveys (36 pre LC and 56 post LC) showed there was a significant increase in staff confidence in providing BF education to mothers (25% vs 46%; p= 0.013) and improved access to LC appointments (5% vs 89%; p0.01).

Conclusions: LC services can significantly improve the provision of breast milk within the first 12 hours of life for very preterm neonates, and improve maternal access to education and support. Further research is required to assess the effect of LC’s on improving long term breast feeding rates.

813

Neonatology

Is Early Oxidative Stress the Common Origin of Both Bronchopulmonary Dysplasia and Impaired Neurodevelopment in Preterm Infants Less Than 29 Weeks of Gestation?

Ibrahim Mohamed , Jean-Claude Lavoie, Francine Lefebvre

¹ Pediatrics/Neonatology and Nutrition, University of Montreal, Canada

Background: Preterm infants are exposed to many oxidants early in life. They have limited antioxidant capacity. Early oxidative stress is involved in bronchopulmonary dysplasia (BPD) development. Studies confirme the association between BPD and impaired neurodevelopmental outcome.

Hypothesis/Objectives: We hypothesis that early oxidative stress has multiorgan negative effects on the lung and the neuro-development. Specific objectives: To examine the effect of early oxidants on the oxidative stress at 36 weeks gestational age (GA) and to test its relationship with both BPD and neurodevelopment.

Design and methods: A prospective cohort study included 51 infants 2 ≥25% on day 7 of life and parenteral nutrition (PN) duration 14 days. t-student test, Chi² and logistic regression were used as appropriate. Baseline characteristics, BPD and Bayley-III at 18 months corrected age were collected. P

Results: The effect of early oxidants on oxidative stress markers at 36 weeks PMA is shown in Table 1.

There was a significant association between exposure to early oxidants and BPD and impaired neurodevelopment Table 2.

Increased oxidative stress at 36 weeks PMA was associated with both BPD and neurosevelopmental impairment Table 3.

In a logistic regression including GA, illness severity score and maternal education; the redox potential at 36 weeks PMA remained a significant independent factor for motor Bayley score (P=0.036) and any Bayley score

Conclusion: Early exposure to oxidants results in oxidative stress that increases the risk of both BPD and neurodevelopmental impairment. We speculate that strategies to re-establish oxidant -antioxidant balance can improve both short and long-term outcomes in infants less than 29 weeks GA.

767

Neonatology

Early Postnatal Automated Spectral EEG Analyses of Premature Infants may Help Predict Neurocognitive Outcome at Adolescence

Tone Nordvik ^1,2 , Eva Margrethe Schumacher ² , Pål Gunnar Larsson ³ , Are Hugo Pripp ⁴ , Gro Christine Christensen Løhaugen ⁵ , Tom Arne Stiris ^1,2

¹ Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Norway

² Department of Neonatal Intensive Care, Ullevål, Oslo University Hospital, Norway

³ Clinical Neurophysiological Section, Department of Neurosurgery, Oslo University Hospital, Norway

⁴ Oslo Centre for Biostatistics and Epidemiology, Oslo University Hospital, Norway

⁵ Department of Pediatrics, Sørlandet Hospital HF, Norway

Background: Early postnatal aEEG/EEG of premature infants

Objective: We have previously demonstrated an association between total absolute band power (tABP) during the first three days of life and developmental outcome at 24 months corrected age in premature infants born before 28 weeks gestational age. The aim of this study is to determine whether this association is still present at adolescence.

Methods: Premature infants with a gestational age 28 weeks were continuously EEG-monitored for the first three days of life. The total absolute band powers of the different frequency bands delta, theta, alpha and beta were analyzed. At 10-12 years of age neurocognitive outcome was assessed using the Wechsler Intelligence Scale for Children 4th edition (WISC), Vineland Adaptive Behavior Scales and Behavior Rating Inventory of Executive Function (BRIEF). The mean difference in tABP was assessed for individuals with normal versus abnormal neurocognitive scores.

Results: 22 infants were included. The infants were assessed at a mean age of 11.5 years of age. tABP in all four frequency bands were significantly lower in infants with pathological results in the main composite score (full intelligence quotient, adaptive behavior composite score and global executive composite score) of all three tests (p0.05).

Conclusion: Early postnatal EEG har the potential to assist in predicting cognitive outcome at 10-12 years of age in extremely premature infants 28 weeks gestation.

542

Neonatology

Excessive Weight Loss in the Perinatal Period: A Case-Control Study

Maria Joao Palha ¹ , Sara Limão ² , Mafalda Crisóstomo ³ , David Lito ² , Mário Paiva ²

¹ Pediatrics, Hospital de Santa Maria, Centro Hospitalar Universitário de Lisboa Norte, Portugal

² Pediatrics, Hospital Vila Franca de Xira, Portugal ³ Pediatrics, Hospital Dona Estefânia, Centro Hospitalar de Lisboa Central, Portugal

Background: Weight loss is expected in the first days of life. There is lack of population-based data on risk factors for newborn’s weight loss.

Objective: Determine factors associated with weight loss higher than 10% at postpartum discharge.

Methods: Prospective case-control study conducted in a second-level hospital during a five-month period, including all the newborns with weight loss 10% at the third day of life. Gestational age less than 35 weeks, birth weight less than 1900g and need for intermediate/intensive care were exclusion criteria. Controls were matched by gestational age, type of delivery and sex. Clinical and demographic variables, and occupancy levels were analyzed. For statistical analysis, we used t-student and qui-square tests (p-value

Results: A total of 59 newborns were included (6.1% of total deliveries). The majority (96.6%) was full-term and 44.1% were delivered via cesarean. Concerning birth weight, no significant difference between groups of cases and controls was found. Mean percentage of weight loss was 11.0% in the case group. There was a higher percentage of gestational diabetes and hypertension during pregnancy in the study group, but the difference wasn’t significant (10.2 vs 1.7%, p 0.051; 8.5 vs 1.7%, p 0.094, respectively). Parents were older in the case group (median age 33 vs 31 years for mothers and 35 vs 33 years for fathers), without significant difference (p 0.294 and p 0.055). We found a significant higher occupancy rate of the nursery (66.8% vs 60.0%) in the group of cases (p 0.012). No significant difference was found regarding number of siblings, maternal active smoking, psychiatric conditions or ethnicity.

Conclusion: Occupancy rate of the nursery was the only factor with a strong association with excessive weight loss in our study, demonstrating the importance of nurse counseling of mothers during the first days of the newborn’s life.

653

Neonatology

Optimal Time to Apply Combined Pulse Oximetry and Physical Examination Aiming in Detecting Severe and Critical Congenital Heart Diseases in Term Neonates

Andreas Petropoulos ^1,2 , Ruslan Huseynov ³ , Nabil Seyidov ⁴

¹ Cardiac ICU, Great Ormond Street Hospital, UK

² Pediatrics-Cardiology, The “Aziz Aliyev” national postgraduate and CME medical training center, Azerbaijan

³ NICU, Neonatal ICU of the Ob/G Institute of Ministry of HealthNICU of Azerbaijan, Azerbaijan

⁴ Childhood Medical Care Sector, Ministry of Health of the state of Azerbaijan, Azerbaijan

Background: Up to date, many studies around the world have proven the use of pulse oximetry(pox) as a screening test with high specificity, moderate sensitivity and a low overall false-positive rate, for detecting critical and severe Congenital Heart Diseases (c/s-CHD). Additional to that important neonatal nosology as acute/chronic lung disease, early onset of sepsis or PPHN can be early detected. Few studies have comment on the exact timing and the amount of measurements needed to obtain the optimal results.

Aim: Prospective screening study by pox + physical assessment, aiming to detect the optimal timing amount of measurements for optimal results

Population-Method: From June 2016 to August 2018, 2570 term neonates were screened in two major maternity units in Baku-Azerbaijan. Pox levels using a motion-tolerant pulse oximeter where blindly reported on the end of 1st and 3ed day of life by a team of trained nurses, using a simultaneous pre and post ductus arteriosus (pre/post DA) measurement. A second team manded by neonatologists then assessed these neonates on the same dates. Firstly, blinded to existing pox measurements and then combining them together with Physical Assessment(pox+PA). Cut-offs of pathological pox were below 90% in any measured site or persisting pre/post DA difference 3%, following 2 assessments within 30 minutes apart. At day five from birth, all of them received Echo-2D screening from two examiners both double-blinded to the results of pox, pox + physician assessment and findings of previous Echo-2D.

Findings: From 2570 screened by Echo-2D, 47 CHD’s were found. 17/47: c-CHD & 5 s-CHD. 25/47 were simple/ moderate complex non-cyanotic CHD.

Pox team; detected 14/22 (63.63%) c-CHD at 24h post-birth. This raised 18/22(81.81%) at 72h post-birth. Also detected 65 cases (2.2%) of additional neonatal non- cardiac pathology.

Combined team: PA only 12/22(55.5%) & 14/ (63.63%). Pox+PA: 17/22(77.28%) & 20/22(90.91%) of c/s-CHD at 24 & 72h post-birth respectable. Additional, 75 cases (3%) neonatal non- cardiac pathology were detected.

Conclusion: Study showed that when the test when applied 72h post birth had the highest positive predicted value screening c/s-CHD test. An additional measurement in the end of first 24h, can determine

559

Neonatology

Multiple Pregnancy: The Experience of A Level III Neonatology Unit

Sara Pinto ¹ , Joana Caldeira Santos ¹ , Lorena Stella ¹ , Ana Marta Pinto ² , Manuela Pinho ² , Márcia Gonçalves ¹ , Manuela Mateus ¹

¹ Neonatology Unit of the Paediatrics/ Neonatology Service, Centro Hospitalar Vila Nova de Gaia/Espinho, Portugal

² Gynecology/Obstetrics Service, Centro Hospitalar Vila Nova de Gaia/Espinho, Portugal

Background: The incidence of multiple gestations (MG) has increased over the last decades, with a consequent increase in the risk of fetal/neonatal morbidity/mortality.

Objective: Evaluate the approach, clinical evolution, complications and follow-up of newborns from MG.

Methods: Retrospective analysis of newborns admitted to the Perinatology Sector (PS) and Neonatal Unit (NU), resulted from MG, between January 2016 and December 2017.

Results: Ninety-eight gestations were identified, 3 triple, and 95 twin. 70.5% were bicorionic/biamniotic. 71% of the pregnancies were older than 31 years and 41% had medically assisted production techniques (APT).

Of the 197 newborns, 52% were female, 73.5% premature (2.7% extreme preterm), and the mean birth weight (BW) was 2062g. 4.5% presented extreme low birth weight (ELBW). 58.4% were admitted directly in NU, 41.6% in PS.

Mean gestational age (GA) of PS group was 36.8 weeks, and BW mean 2569.7g. 52.9% had hospitalization longer than protocolated.

Median length hospitalization`s in NU was 19 days, with 77.2% requiring intensive care. Mortality rate was 2.5%, with 80% being extreme premature.

83.7% had a follow-up visit, with a poor staturo-ponderal evolution in 2% and inadequate psychomotor development in 6%.

Extreme prematurity was associated with mortality (p0.001), and intraperiventricular hemorrhage (IPVH) (p0.001). Need for surfactant and mechanical ventilation were correlated with low birth weight (p0.001,p=0.002), very low birth weight (VLW) (p0.001,p=0.027) and ELBW (p0.001,p0.001).

Need for intensive care was associated with ELBW (p=0.006) and VLW (p0.001). Inadequate psychomotor development was associated with VLW (p=0.036), and inadequate staturo-ponderal development with intrauterine growth restriction (p=0.001).

Conclusion: As described in the literature, maternal age was high and there was a high percentage of APT use.

In addition to complications inherent to prematurity, we found an extension of PS hospitalization time.

BW and GA are important risk factors for neonatal IPVH, respiratory morbidity, inadequate psychomotor development and mortality.

683

Neonatology

Maternal Adipokines in Relation to Newborn Weight

Maysa Saleh ² , Enas Rafaat ² , Yasmine El Gendy ¹ , Lobna Sherif ² , Hanaa Hamdy ³

¹ Pediatrics, Ain Shams University, Egypt

² Child health, NRC, Egypt

³ Hormone, NRC, Egypt

Background: Intrauterine exposure and its relation to the offspring metabolic and growth outcome is not clear .

Adipokines; leptin and adiponectin are correlated with adult and childhood obesity, yet early gestational exposure and its relation with offspring growth needs to be determined.

Objectives: To assess the relation between serum maternal and umbilical cord blood level of adipokines with the newborn weight.

Subjects and Methods: 50 pairs of mother and their newborns (N:100) were enrolled in the study. Serum maternal and umbilical cord adipokines ( leptin and adiponectin) level were determined. Maternal and their newborn anthropometric measurement were obtained by trained examiners.

Results: Lower serum maternal and cord blood level of leptin are associated with higher newborn weight compared to appropriate for gestational weight ( p0.05). Moreover increased newborn weight was associated with higher level of cord blood adiponectin (p0.05).

cord blood of leptin was significant increased in newborns whose mother were obese when compared to normal weight mothers (p0.05).

Conclusions: Maternal adipokines level affects the newborn weight

602

Neonatology

Effects of Probiotics on Controlling Neonatal Jaundice

Devajit Kumar Sarma ¹

¹ Neonatology, Satribari Christian Hospital, India

Background: Hyperbilirubinemia remains the commonest cause of hospitalisation and readmission of new-borns. It can also cause considerable morbidity and mortality if not treated timely and properly.

Objectives: The aim of this study is to assess the effect of probiotics on controlling neonatal jaundice.

Methods: 102 consecutive born new-borns, beyond 32 weeks of gestations who met inclusion criteria, were randomly assigned to either control or pro-biotic group. Those in probiotic group ( group I) were given once daily dose of probiotic with Bacillus clausi, 2 billion unit and exclusive breastfeeding from day 1 until day 7. The control group (group II) were only on breastfeeding. Serum bilirubin was estimated on all babies on day 2, 4 and 7.

Results: There were 51 infants in each group. Out of which 24 babies in the probiotics group and 27 babies in the control group were in high-risk categories for neonatal jaundice. On day 2 bilirubin on the group I was 6.5 mg/dl +/-1.7, and in group, II was 8.6 mg/dl +/- 2.6 ( P=0.0001). Similarly on day 4 it was 10.2 +/- 2.3 group I and 12.1+/-3 in group II( P= 0.0005) and 8.6 +/-2.6 and 97 +/- 3.4 on day 7 ( P=0.983)

An innovative contribution to research-

Probiotics are effective in controlling neonatal jaundice and subsequent need for hospitalization.

643

Neonatology

Comparision of Gradual Versus Sudden Weaning of Preterm Neonates from Nasal CPAP

Harshal Wagh ¹

¹ Pediatrics, Bharati Vidyapeeth (Deemed to be) University, Medical College & Hospital, Sangli, India

Background: Advantages and disadvantages of CPAP are well known but ideal strategy of weaning from CPAP is still unknown. Removing neonate from CPAP may lead to increased O₂ requirement, increased work of breathing, need to restart CPAP. Hence safe weaning methods are required to prevent neonatal mortality and morbidity.

Objective: To compare gradual versus sudden weaning of preterm neonates from nasal CPAP.

Methods: Preterm neonates (37weeks, 2.5kg) on CPAP were included; those with major malformations & air leaks were excluded. Neonates ready to be weaned were randomized. If the neonate was in ‘sudden’ weaning group, the baby was directly kept off CPAP for next 72 hours. If the neonate was in ‘gradual’ weaning group, the neonate was kept off CPAP for predetermined hours each day. All babies were closely monitored. If the neonates developed distress while weaning, they were put back on CPAP.

Results: Out of 91 neonates, 46 (50.5%) were weaned by sudden weaning, while 45 babies (49.5%) were weaned gradually. Among the 77 babies (84.6%) who succeeded weaning, 44 (48.3%) were from the gradual weaning group as compared to 33 (36.2%) in the sudden weaning group. The chi-square statistic was 11.8476 (p 0.05). Failure rate was more (14.2%) in the sudden weaning group (p 0.05). Success rate was more (58.2%) in babies with birth weight 1.5-2.5kgs and gestational age of 32-37 weeks (58.2%) (p value 0.01).

Conclusions: Gradual weaning was found to be better than sudden weaning. Success was more in babies 1.5 kg & 32 weeks.

249

Nephrology

Primary Hyperoxaluria Type 1: A Case Report

Ines Ganhao ¹ , Catarina Borges ² , Telma Francisco ³ , Marta Amorim ⁴ , Isabel Gonçalves ⁵ , Margarida Abranches ³

¹ Serviço de Pediatria, Centro Hospitalar Barreiro-Montijo, Portugal

² Pediatria, Unidade Local de Saúde do Baixo Alentejo, Portugal

³ Unidade de Nefrologia Pediátrica, Centro Hospitalar Universitário de Lisboa Central, Portugal

⁴ Serviço de Genética, Centro Hospitalar Universitário de Lisboa Central, Portugal

⁵ Unidade de Hepatologia, Centro Hospitalar e Universitário de Coimbra, Portugal

Background: Primary Hyperoxaluria type 1 (PH1) is a rare autosomal recessive inherited disease, with an estimated incidence of 1:100 000 live births per year in Europe. It is caused by mutations in AGXT gene, leading to liver-specific peroxisomal enzyme alanine-glyoxylate aminotransferase (AGT) dysfunction. Its heterogeneous presentation proves a diagnostic challenge and a high level of suspicion is necessary for an early diagnosis. Over 50% already present with end stage renal disease (ESRD) at time of diagnosis.

Case report: We report the case of a 14-year-old boy, second child to consanguineous parents, followed in Pediatric Nephrology consultation of a tertiary hospital. No relevant past history, except for enuresis and minor beta-talassemia. He was first referred at the age of 9 for recurrent lithiasis (episodes of stone elimination) and ureteral dilatation. At presentation, estimated glomerular filtration rate (eGFR) was 32.3 mL/min/1.73m² and ultrassonography (US) confirmed bilateral renal lithiasis causing right ureter obstruction and nonspecific parenchymal hyperechogenicity. Stone analysis revealed calcium oxalate monohydrate crystals and 24-h urine had markedly elevated urine oxalate excretion (2.22 mmol/1.73m²/day). Besides hydration, he was treated with sodium and potassium citrate and pyridoxine, and eGFR transiently improved (93.7 mL/min/1.73m²). However, despite increasing doses, urine oxalate excretion remained more than 3-fold higher than reference values. Genetic tests confirmed a mutation in AGXT gene, c.1151TC, in homozygosity. Approximately two years after initial symptoms, nephrocalcinosis was identified in US, consistent with the diagnosis, and eGFR gradually declined while albuminuria and uricemia got elevated. Oxalate deposition in myocardium, retina, bone marrow and thyroid was excluded following a multidisciplinary approach. At 14 years old, eGFR went bellow 40 mL/min/1.73m² and he was then proposed for a sequential liver-kidney transplantation. Liver transplantation of deceased donor was performed with no major complications.

Conclusions: Recurrent urolithiasis and nephrocalcinosis in children along with suggestive family history/consanguinity should raise the suspicion of PH1. Definite diagnosis is based on mutation analysis. Conservative treatment may increase renal survival, therefore it should be started when the disease is first suspected. Sequential or combined liver and kidney transplantation should be planned when eGFR declines below 40mL/min/1.73m² to avoid systemic complications of systemic oxalosis.

562

Neurology

Pediatric Care of Neurodevelopmental Disorders

Maureen Casper ¹

¹ Speech-Language-Hearing Sciences, City University of New York, USA

Background: Differential diagnosis and monitoring of treatments for children with neurodevelopmental disorders continue to present a challenge in pediatric care. Deficits in the control of coordinated movement of limb, speech and voice production often present in those children diagnosed within the spectrum of neurodevelopmental disorders. The measurement of speech and voice production allows for a sensitive and quantitative description of coordinated movement. Vocal fold vibration and movement of the articulators may be quantitatively assessed with acoustical measures of fundamental frequency (F0) and formant frequencies (F1 and F2) as well as syllable duration. When embedded within a stimuli designed to elicit prosodic contrasts (Casper, et al., 2006) these measures provide an objective index for monitoring speech motor control.

Objective: Normal child development is marked by intelligible speech, gradual increase in length and complexity of utterances and flexible prosody. Both standard assessment and acoustic analyses of speech are reviewed as measures for evaluating neurological status.

Method: Differential diagnosis and treatment protocols directed at improving speech motor control were used to assess and monitor neurological status in pre-school children aged 3-5 years. Case studies presented evaluate verbal apraxia, sensory processing and delayed language development.

Results and Conclusion: The results of these analyses and the other uploaded clinical data provide objective measures for the rate of progress of a child diagnosed within the spectrum of neurodevelopmental disorder in the areas of intelligibility, length and complexity of utterances, and speech prosody. The data and results can be shared across physicians, clinicians and researchers to improve the efficacy of treatment protocols and identify areas of intervention that need emphasis in developing the child’s neurophysiological ability to produce and perceive spoken language.

142

Neurology

Epidemiology of Autism Spectrum Disorders: A Multifactorial Model for Populations in Transition

Gary Diamond ^1,2 , Lutfi Jaber ^1,2,3 , Ditza Tsahor ⁴

¹ Pediatric Neurology and Child Development, Schneider Children's Medical Center, Israel

² Pediatrics, Clalit Health Services, Israel

³ Pediatrics, Bridge to Peace Community Health Center, Israel

⁴ Autism Center, Asaf Harofeh Medical Center, Israel

Background: Autism Spectrum Disorders (ASD) are a group of developmental disabilities characterized by impairments in social communication, restricted areas of interest, and repetitive and stereotyped patterns of behavior. Symptoms typically are apparent by the age of 3 years.

Objective: To determine the prevalence of ASD among children of the largest of 4 health maintenance organizations in Israel.

Methods: Point prevalence and diagnostic trends of ASD over the past 18 years were examined over a wide geographical area of central Israel, encompassing a database of 331,169 children, age 3-18 years. Special importance was attached to statistics from different religious and ethno-cultural groups as potentially reflecting discrepancies in diagnosis, reporting, and possibly environmentally-related trigger factors in the causality of an essentially genetically determined syndrome.

Results: Overall prevalence was 0.004 (1/250 live births), well below firgures from other similar studies abroad and in Israel. Prevalence figures for the ultra-orthodox Jewish community were the lowest (0.0022), when compared with the general population and Israeli Arabs (0.0033). Time trends indicated a surge of ASD among Israeli Arabs and Ethiopian Jews between the years 2004 and 2015, in contrast with a general flattening for the ultra-orthodox community (p0.01).

Conclusions: Results indicated that besides discrepancies in diagnosis and reporting factors, there exists a possible relation between clinical expression of an ASD genetic predisposition and the socioeconomic/ cultural environmental factors at work in impacting on the epigenetics in the causality of autism. Our prevalence rates are currently lower than those for ASD in Europe and the USA. The results should be of special interest to planners of health and educational services for the handicapped in countries with minorities or immigrant groups in social transition.

479

Neurology

Nusinersen: a Single-Center Real-Life Experience in Type 1 Spinal Muscular Atrophy

Andreia Lomba ^1,2 , Joana A. Ribeiro ² , Henriqueta Araújo ² , Vera Ribeiro ² , Núria Madureira ³ , Filipe Palavra ^2,4 , Isabel Fineza ²

¹ Pediatrics Department, Centro Hospitalar do Baixo Vouga, Portugal

² Centre for Child Development - Neuropediatrics Unit, Hospital Pediátrico, Centro Hospitalar e Universitário de Coimbra, Portugal

³ Pediatrics Department – Pneumology Unit, Hospital Pediátrico, Centro Hospitalar e Universitário de Coimbra, Portugal

⁴ Faculty of Medicine, Coimbra University, Portugal

Background: Nusinersen, an antisense oligonucleotide designed to treat 5q-Spinal Muscular Atrophy (SMA), is currently available in Portugal for SMA patients. In clinical trials, SMA type 1 patients achieved a substantial motor milestone response.

Objective: To report the clinical results of nusinersen in a single tertiary centre, in Portugal.

Methods: We performed a retrospective study of type 1 SMA patients treated with nusinersen. Demographic, clinical (including motor scores – CHOP-INTEND), ventilation and feeding parameters were obtained from clinical records.

Results: We are currently treating 5 genetically confirmed SMA type 1 patients – current mean age 16,4 months (range 8–25 months), four with 2 copies of SMN2 and one with 3 copies of SMN2. The mean time from the first symptoms to the diagnosis confirmation was 3,1 months (range 0,3-10,0) and from the diagnosis confirmation to the beginning of treatment was 1,1 months (range 0,5-1,7). Only 3 patients had ≥ 6 months of treatment (5, 6 and 9 administrations of nusinersen respectively), and they all improved at least 4 points in CHOP-INTEND score (mean change 10.0 points). The eldest patient (25 months-old) began treatment at 2,3 months and has achieved independent sitting. Prior to treatment, 2 patients required continued non-invasive ventilation (NIV) and one 14 hours per day. After 6 months of treatment they all reduced time of NIV up to 8 hours per day. Gastrostomy was performed in 2 patients during treatment and one required tube feeding. No side effects of treatment were noticed.

Conclusion: We observed an improvement in motor function and ventilatory support in SMA type 1 patients with ≥ 6 months of treatment with nusinersen. Despite our small sample, our findings are promising and contribute to the increasing evidence that early diagnosis and treatment is paramount for these patients.

401

Neurology

PANS as a Post-Infectious Autoimmune Disease: Benefit of IVIG

Isaac Melamed ¹ , Andrew Schechterman ¹

¹ IMMUNOe Research Center, IMMUNOe Health Centers, USA

Background: Pediatric acute-onset neuropsychiatric syndrome (PANS) is a clinical diagnosis given to children who have a dramatic – almost overnight – onset of neuropsychiatric symptoms. Recent findings suggest a relationship between a post-infectious response and behavioral changes, which may be a form of post-infectious autoimmunity, through molecular mimicry, resulting in various neurological symptoms. We’ve coined the term “Alzheimer’s of the Immune System,” or AIS, to identify this syndrome. As a result of our studies and observations, we’ve identified a number of common immune defects in patients with this presentation which suggests a new immune deficiency. Given our findings, we hypothesize that AIS is the underlying mechanism that leads to PANS.

Methods: Based on this hypothesis, we proposed a study to explore the use of intravenous immunoglobulin (IVIG)[Octagam 5%] for the treatment of PANS. The primary objective of the study was to assess the efficacy of IVIG in PANS over a period of 6 months (6 infusions) based on psychological evaluation scores using 6 different assessments (Figure 1). Secondary objectives included evaluation of the immunomodulatory effect of IVIG on key biomarkers associated with PANS.

Results: Our results indicate statistically significant reductions in symptoms from baseline to end of study (infusion 6) in all 6 assessments measured. Dramatic results can be seen in the PANSS-PI (Figure 2). Statistically significant reductions in symptoms were demonstrated beginning at infusion 3 through infusion 6 compared to baseline, with steady improvement from infusion 1 to infusion 6. Additional results from CY-BOCS indicated statistically significant reductions in obsessive compulsive symptoms, resulting in 50% improvement, that were sustained 8 weeks after the final infusion (Figure 3).

Conclusion: In patients presenting with PANS, which may be associated with an underlying immune deficiency mechanism we term AIS, IVIG [Octagam 5%] successfully mediated psychological symptoms and dysfunction.

144

Neurology

Long Term Neurodevelopmental Outcome following Therapeutic Hypothermia for Perinatal Asphyxia

Basavaraj Patil ¹ , Pranay Boralkar ¹ , Sharanagouda Patil ¹ , Roopa Mangshetty ¹ , Rohit Bhandar ¹ , Apurva Arun ¹ , Kudlappa Angadi ¹ , Mallikarjun Kobal ¹ , Kiran Hosgouda ¹

¹ Pediatrics, Mahadevappa Rampure Medical College, India

Introduction: Neuroprotective benefit of therapeutic hypothermia in term newborns with hypoxic-ischemic encephalopathy (HIE) was analyzed by survival and neuro-developmental outcome of neonates subjected to this procedure.

Material and Methods: Newborns with gestational age 36 weeks and 6 hours of age with moderate to severe asphyxial encephalopathy underwent cooling protocol at a temperature of 33.5 °C for 72 hours and rewarming period of 6 hours. Outcome measures assessed were death and neurodevelopmental characteristics, which were compared at the different age using ASQ3.

Results: 42 children were assessed at age 6-8months, 42 children at 12-14months. Median gestational age was 38 weeks, birth weight 2.8kg, Apgar score 2/4 and pH on admission to the hospital 7.02. Four (8%) children died. At the first assessment developmental categories of communication were normal in 61.9%, problem solving in 69.1%, personal-social in 61.9%, gross motor in 66.27%, and fine motor in 40.5% with a high need of retesting in this area. Second assessment was done in 42 patients: developmental categories of communication normal in 78.6%, problem solving in 76.2%, personal-social in 71.4%, gross motor in 81.0%, and fine motor in 50%.

Conclusion: There was no correlation between baseline parameters and outcome. Results of the study are showing that therapeutic hypothermia in term newborns can provide better survival and less neurologic sequels in HIE patients.

Table Baseline data at birth for patients enrolled in study

				Range
Gestational age-weeks	38.6	1.4	40	37-42
Birth weight-grams	2800	450	3.25	2.5-4
Head circumference-cm	35.12	1.1	35	33-37
Apgar score at 1min	2.26	1.772	2	0-6
Apgar score at 5min	4.37	1.363	4	2-6
Ph at hospital admission	6.99	0.186	7.02	6.5-7.3
Age at starting of cooling-hours	4.32	2.33	4	2-8

157

Obesity

Obesity in Relation to Hypertension in a Sample of Egyptian Adolescents with Insulin Resistance

Moushira Zaki ¹ , Sanaa Kamal ¹ , Karima Bahgat ² , Walaa Basha ¹

¹ Biological Anthropology, National Research Centre, Egypt

² Pediatric, Faculty of Medicine, Al-Azhar University, Egypt

Background: Obesity is a global epidemic disease that goes worse in many countries all over the world and has very serious effects on health status and economy of the community.

Objectives: The aim of this study is to evaluate the association between obesity and high blood pressure levels in a sample of obese Egyptian adolescents with insulin resistance (IR).

Methods: A cross-sectional study was conducted on 200 obese adolescents with (IR), aged between 16 and 17 years (100 males and 100 females) and 200 non-obese healthy controls matched in age and sex. Anthropometric parameters, body composition, fasting insulin and glucose were measured. IR was evaluated by the Homeostasis Model Assessment-Insulin Resistance (HOMA-IR). Spearman correlation and multiple regression analysis were used to assess the relationship. Receiver operating characteristic curve (ROC) analysis was performed to obtain the best cut-off values, sensitivity, and specificity for discrimination of hypertension in obese IR.

Results and Conclusions: Results show positive correlations between systolic and diastolic blood pressure, HOMA-IR, body mass index (BMI), waist circumference and body fat%. Multiple regression analysis showed that BMI, waist circumference, waist to hip ratio and body fat percentage were independently related to systolic and diastolic blood pressure in IR patients in both genders, suggesting the importance of their use in the evaluation of hypertension among obese adolescents with IR.

328

Obesity

Association between Overweight/Obesity, Serum Gonadotropin and Androgen Levels among Egyptian Pubertal Girls

Sahar El-Masry ¹ , Rehab A. Mahmoud ³ , Muhammad Al-Tohamy ¹ , Hanaa H. Ahmed ² , Mahmoud A.S. Afify ¹

¹ Biological Anthropology, National Research Centre, Egypt ² Hormones Department, National Research Centre, Egypt ³ Pediatrics Department, Institute of Postgraduate, Childhood Studies, Ain Shams University, Egypt

Background: Previous studies have suggested an association between adiposity and elevated androgen in late pubertal girls.

Objective: Evaluate the association between overweight /obesity, serum gonadotropin and androgen levels among Egyptian pubertal girls.

Subjects and Methods: It was a cross-sectional one, carried out in the Obesity Clinic of the Diabetes, Endocrine and Metabolism Pediatric Unit (DEMPU), Pediatric Hospital, Cairo University. It included 40 overweight and obese girls and 40 age-matching normal weight (control) girls, aged 12 -18 years. Anthropometric assessment (weight, height, hip and waist circumferences) was recorded, and waist/hip and BMI were calculated. Laboratory investigations: lipid profile. (TG, TC, HDL, LDL), serum gonadotropin (LH, FSH), androgen (free and total testosterone), estradiol, insulin, HbA1c, and fasting blood sugar were measured .Insulin resistance (IR) was calculated.

Results: Hypogonadotropins (FSH and LH) and hyperandrogenaemia (total and free testosterone) were significantly prominent among obese girls. Correlation between gonadotropin (FSH and LH), androgen (Total Testosterone and free Testosterone) and all the studied variables for the 3 groups under study (obese, overweight, control) revealed constant relations. Gonadotropin and androgens had opposing correlations. Gonadotropin had significant negative correlations, and androgens had significant positive correlations with the anthropometric obesity markers (BMI, Waist C, and W/H ratio), HbA1c, insulin, insulin resistance and lipid profile (triglycerides, total cholesterol and LDL). In addition, Gonadotropin had significant positive correlations, and androgens had significant negative correlations with estradiol and HDL, and insignificant correlation with age.

Conclusion: Overweight and obesity had no effect on the correlations between gonadotropin and androgen in one side and the anthropometric measurements and laboratory investigations on the other side. The changes in androgen levels were earlier than in gonadotropin among the overweight and obese children.

186

Obesity

Prevalence of Overweight and Obesity in Youth (7-21 Years) with Intellectual Disabilities, without Down Syndrome, in the State of Kansas, USA: 2012-2019

Edwin Loovis ¹ , Ken Pitetti ² , Ruth Ann Miller ³

¹ Department of Health and Human Performance, Cleveland State University, USA ² Department of Physical Therapy, Wichita State University, USA ³ Department of Electrical Engineering, Wichita State University, USA

Background: For typically developing (TD) youth in the United States (USA), Odgen and colleagues (2014) reported that for combine sexes the prevalence of overweight (OW) was 34.2% and 34.5% in ages 6-11 years and 12-19 years, respectively; and prevalence of obesity (OB) (Ogden and colleagues, 2016) of 17.4% and 20.5% in ages 6-11 years and 12-19 years, respectively. However, there has been no tracking of OW and OB rates of youth with intellectual disability (ID) in the USA.

Objective: To provide current estimates of the prevalence of OW and OB of youth with ID, without Down syndrome (DS), for ages 7-21 years in a midwestern state (Kansas).

Methods: Two hundred and twenty-two (222) males and 108 females, ages 7-21 years with moderate to mild ID without physical disabilities were evaluated between 2012 and 2019. Height and weight were measured using standardized protocols and body mass index (BMI: wt[kg]•ht[m]^-2) was calculated for age groups 7-12 years (children); 13-16 (adolescents); 17-21 (young adults). BMI classifications of OW and OB were determined using international standards for age and sex specific cut-off points (Cole et al., 2000).

Results: Results are illustrated in Table 1.

Table 1. Prevalence of OW and OB by Age and Gender

Overweight Obese 7-12 yrs 13-16 yrs 17-21 yrs 7-12 yrs 13-16 yrs 17-21 yrs
M	20%	18%	25%	15%	30%	34%
F	13%	31%	36%	17%	21%	40%
M+F	17%	26%	27%	15%	29%	34%

Conclusion: Compared to TD youth in the USA: youth with ID in Kansas reported lower prevalence of OW and OB in ages ≤12 years; and a lower prevalence of OW and a higher prevalence of OB in adolescents and young adults, respectively. These results challenge previous reports that OW and OB are more prevalent in populations with ID, without DS, and suggest that regional differences in obesogenic environments may exist.

480

Pediatric Cardiology

Changes in the Cardiovascular System during Therapy with Propranolol of Infantile Hemangiomas

Nataliya Konstantinova ¹ , Natalia Kotlukova ^1,2 , Ekaterina Karelina ² , Aleksei Grishkin ² , Tatiana Lavrova ³

¹ Tabolin Department of Hospital Pediatrics, Pirogov Russian National Research Medical University, Russia ² Department of Pediatric Cardiology, Bashlyaeva Children's City Clinical Hospital, Russia ³ functional diagnostics department, Bashlyaeva Children's City Clinical Hospital, Russia

Background: Propranololol is undeniably effective as an infantile hemangiomas (IH) treatment, however, the drug acts on the myocardial tissue and heart rate.

Objective: To assess the state of the cardiovascular system in patients before treatment, during therapy with propranolol and after its completion.

Methods: Cardiac examination (ECG, echocardiogram, 24-Hour Holter monitoring), and ultrasound with Doppler of IH vessels were performed 202 children aged 1 month to 4.5 years with IH different localization. Studies were conducted before the appointment of propranolol therapy, then every 3 months during treatment and after discontinuation of therapy.

Results: According to the ECG, there was not a single case of bradycardia. However, 24-Hour Holter monitoring showed that in all children, 3 months after the start of therapy, the average daily heart rate decreased by 9% from the norm. Also, according to 24-Hour Holter monitoring, 18% of children were diagnosed with AV-blockade of grade I-II and 6% of children were diagnosed the rhythm pauses, significantly exceeding the age norm. Syncopal states were observed in 2 children, accompanied by pauses of the heart rhythm with a maximum duration of 18 seconds. In 1 child, according to the echocardiogram, there was a decrease in the contractile function of the myocardium. Doppler ultrasound found that when blood flow in the vessels was

Conclusion: Cardiological examination revealed a small percentage of undesirable effects on the background of propranol. Only a flow-up 24-Hour Holter monitoring allowed timely identification of life-threatening changes in heart rate and timely correction of the dose of the drug. Ultrasound with Doppler allowed us to establish an individual duration of treatment with propranolol and to minimize the percentage of rebound syndrome against the background of discontinuation of therapy with propranolol.

672

Paediatric Cardiology

Postnatal Echocardiographic Screening of Babies with Positive Family History of Congenital Heart Disease. A Two-Year Retrospective Study

Rachel Hoi Khoon Tan ¹ , Hossam Khaliel ¹ , Maysara Aziz ¹

¹ Paediatrics Department, St Helens and Knowsley Teaching Hospitals NHS Trust, UK

Background: Congenital heart disease (CHD) is the most common congenital disorder in newborn. The incidence is 1%. There is good evidence that left ventricular outflow tract obstructions have ‘high heritability’, however there is no consensus guidelines on screening babies with positive family history. Often the family history is vague and unclear.

Aim: To review the family history, and detection rate of CHD, for the babies with family history of CHD.

Method: Retrospective review of two-year data on all babies who were referred to clinic with family history of CHD. The data collected include: gender, pre- and post-ductal saturations, the types of family history, family affected by CHD, investigations performed and the findings on referred babies.

Results: Out of 665 of new patients seen in cardiac clinic between January 2017 to December 2018, 75 (11%) babies were referred for family history of CHD. Only 69% of these babies have pre- and post-ductal saturations done, and all of them were normal. Heart murmur presented in 20 babies (27%).

The most common family history is ‘hole in the heart’, and unspecified CHD. And eight families only declared history of heart murmur. The CHD was most commonly presented in siblings (29%), followed by mother (20%) and father (20%).

9 out of 75 (12%) of these babies were found to have abnormal findings on echocardiogram. This is a 10-fold increased in risk if compared to general population. Most common CHD detected is ASD. Out of them, two babies (22%) have heart murmur on clinical examination.

Conclusion: Obtaining accurate cardiac family history during newborn check can be challenging and baby who has CHD is often asymptomatic. Screening of babies with family history of CHD is justified, but may need to be more targeted, in light of new studies on inheritability of CHD.

856

Paediatric Tropical Medicine

Rotavirus Vaccine, Knowledge and Practice of Parents and Caregivers in a Teaching Hospital in Southeast Nigeria

Chikosolu Okiche ¹ , Chineze Okorowo ¹ , Lilian Nwobashi ¹

¹ Paediatrics, Federal Teaching Hospital Abakaliki, Nigeria

Introduction: Diarrhoeal disease is the second leading cause of death in children under five years old. Rotavirus and Escherichia coli, are the two most common etiological agents of moderate-to-severe diarrhoea in low-income countries. Therefore, rotavirus vaccination is pivotal in the prevention of severe forms diarrhoea disease. The vaccine is yet to be adopted to be given for free in Nigeria and costs about $20 (N7, 000) per dose.

Objectives: This study, which is a pre-intervention study, aimed at understanding the knowledge, perception and practices of parents about rotavirus vaccination and also educate the parents about the vaccine in a tertiary hospital setting in an Eastern State of Nigeria

Methods: In a descriptive cross-sectional study, 186 caregivers of children who came to the children outpatient clinic were enrolled. A researcher administered questionnaire was used to assess the knowledge and practice of parents about rotavirus vaccine. The parents who did not know about the vaccine where also educated on the vaccine and when it’s given.

Result: The mean age of respondents was 33.4years, 83% were mothers and the rest fathers. On knowledge, 76.9% of the respondents didn’t know about rotavirus vaccine. Also as regards practice, 91.9% of the respondents did not vaccinate their children and of them, 21% did not vaccinate because of the cost of the vaccine. 152 (81.7%) of parents said they will like to vaccinate their children and 64.5 % agreed to do so even if they will have to pay for it with only 36.6% of the parents saying they will not be willing to pay

Conclusion: Majority of the parents didn’t know about the rotavirus vaccine as a means of preventing diarrhoea and were excited to hear about the vaccine, its cost and when it is given. This emphasizes the need for more awareness about the vaccine.

241

Paediatric Tropical Medicine

Severe Acute Malnutrition (SAM): A Vicious Socio Economic Problem in South India

Basavaraj Patil ¹ , Pranay Boralkar ¹ , Sharanagouda Patil ¹ , Roopa Mangshetty ¹ , Apurva Arun ¹ , Rohit Bhandar ¹ , Kiran Hosgouda ¹ , Mallikarjun Kobal ¹ , Kudlappa Angadi ¹

¹ Pediatrics, Mahadevappa Rampure Medical College, India

Introduction: Out of 19 million SAM child in all developing countries, 8 million (42%) are in India.

Materials and Methods: Study type: Observational. Study design: Observation model – cohort, Time perspective-prospective. Study centre: Basaveshwar and Sangameshwar teaching hospital. Study duration: august 2017 -september 2018. Informed consent was obtained from the parents of all the children.

Aims and Objectives: To evaluate the clinico-epidemiological profile, complications, and comorbidities of SAM children. To identify the socio-demographic risk factors of SAM and to evaluate the outcome of hospitalized SAM children.

Results: Mean age of admitted children was 17.21±13.94 months and mostly belonged to lower socio-economic scale, rural areas and large families. 9.2% were less than 6 months at time of admission. Male: female ratio was 1:1.24. Most common associated infections were acute respiratory tract infections (35.27%), and diarrhoea (31.75%). oAnaemia was an important co-morbidity (83.52%). Mean duration of exclusive breastfeeding was 3.1± 1.8 months and mean age of starting semi-solid complementary food was 8.5±2.5 months. Most commonly used supplementary food was over diluted cow milk (92%). Cure rate was 75.24%. Defaulter rate was 13.65% and death rate was 0.63%.

Conclusion: The problem of SAM is multidimensional and tertiary care centres provide life-saving care for children as demonstrated by the high survival rates.

CHARACTERISTICS OF SAM CHILDREN

					48-60MO
8	26	31	11	6	3

LITERACY STATUS OF MOTHER

		PERCENATGE
ILLITERATE	35	41.1
PRIMARY & MIDDLE SCHOOL	30	35.2
HIGH SCHOOL	19	22.3
GRADUATE &POST GRADUATE	1	1.1

ECONOMIC STATUS OF SAM CHILDREN

		PERCENTAGE
UPPER	0	0
UPPER MIDDLE	2	2.35
LOWER MIDDLE	3	3.52
UPPER LOWER	30	35.29
LOWER	50	58.81

AWARENESS OF MALNUTRITION

		PERCENTAGE
YES	21	24.7
NO	64	75.29

IMMUNISATION STATUS

		PERCENTAGE
UNIMMUNISED	10	11.75
PARTIALLY IMMUNISED	70	82.35
FULLY IMMUNISED	5	5.84

RESIDENCE(URBAN/RURAL)

		PERCENTAGE
URBAN	21	25
RURAL	64	75

378

Primary Care

Parental Beliefs and Attitudes towards Pediatric Vaccination in a Country with High Vaccine Coverage

Luisa Barros ¹ , Inês Cabral Fonseca ¹

¹ Faculdade de Psicologia, Universidade de Lisboa, Portugal

Background: Anti-vaccination movements are rising in different European and Western countries, causing a decline in the rate of pediatric vaccination. Portugal has one of the highest vaccine coverage rates among countries with non-mandatory pediatric vaccination, and overall higher levels of vaccine confidence. However, anti-vaccination ideas are spreading among parents of young children.

Aim: This study aimed to characterize parental beliefs and attitudes towards vaccination in Portugal and to explore its association with vaccine hesitation.

Methods: Based on the relevant literature, we developed a questionnaire to assess vaccines` safety and efficacy perception, perception of severity of vaccine-prevented diseases, conspiracy theory ideas, beliefs about immunizations requirements, social norms and relationship with health professionals. This questionnaire was disseminated online using the snowball method and several parenting social media groups. Parents of children 0-12 years old were invited to participate.

Results: The questionnaire showed good validity and reliability. A sample of 1118 parents aged 18 to 58 years old filled the questionnaire, and only 3.1% reported vaccine hesitancy regarding an eventual future child. All the evaluated dimensions showed discriminant power. A binary logistic regression was performed to test for discriminative power and predict vaccine hesitation. Perception of vaccine`s safety and efficacy, and positive attitudes towards vaccination requirements predicted the intention to adhere to the recommended immunization plan in the event of having another child.

Conclusions: The large majority of Portuguese parents of young children hold vaccine-favorable beliefs and attitudes. All dimensions evaluated were significantly different for parents who would not vaccinate a new child compared to those who would. Beliefs regarding vaccine`s safety and efficacy and acceptance of school or health services requirements were the most relevant dimensions for this choice.

385

Primary Care

Feasibility Testing of the Core Set of Quality Indicators for Paediatric Primary Care in Europe, COSI-PPC-EU

Dominik A. Ewald ¹ , Gottfried Huss ² , Rike Antje Kraska ³ , Max Geraedts ⁴

¹ Health Services research, German Society of Primary Care Paediatrics, Germany

² Health Services research, European Association of Primary Care Paediatricians, ECPCP, Germany

³ Institute for Health Systems Research, Faculty of Health, Witten/Herdecke University, Germany

⁴ Institute for Health Services Research and Clinical Epidemiology, Faculty of Medicine, University of Marburg, Germany

There is a need to measure and improve the quality of paediatric primary care in Europe where major differences in the delivery and outcomes of child health care exist. A collaborative panel of paediatric senior experts developed a Core Set of Indicators for Paediatric Primary Care in Europe by compiling 42 quality indicators in a modified consensus process following the RAND/UCLA appropriateness method. The aim of this study was to explore the feasibility of the quality indicator set in European paediatric primary care practices. Seventy-nine practices from eight countries participated in a detailed online interview. The practices rated the applicability, relevance, reliability and acceptance of the 42 quality indicator as well as the availability, technical feasibility and effort to retrieve the needed data from their medical records. Most quality indicators were considered applicable, available, reliable, acceptable and relevant for monitoring quality of care in paediatric primary care. Respondents rated feasibility and effort to retrieve the data lowest because of difficulties collecting the data from the medical records.

Conclusion: European paediatric primary care practices generally agree with the proposed quality indicator set. They document most of the parameters. However, the collection of specific needed values from available routine patient-data is considered technically difficult and time-consuming.

749

Primary Care

Early Predictors in Infancy for a Developing Neurobehavioral Disorder – a Pilot Study

Mina Gurevitz ¹

¹ Pediatrics, Maccabi Health Services, Israel

Background: The increasing incidence of Neurobehavioral Disorders (NBDs), such as Autism Spectrum Disorder (ASD), Attention Deficit Hyperactivity Disorder (ADHD) and Developmental Coordination Disorder (DCD), is of major concern in public health. Although early detection of a developing NBD is critical to enable intervention while brain plasticity is prominent, no screening tool for NBD development at infancy is available.

Objective: The aim here was to assess whether risk factors that predict forthcoming NBDs can be identified at early infancy.

Methods: The Israeli Health system provides a general developmental screening system from birth through preschool years at special clinics. In a retrospective cohort study the charts of 161 toddlers, who were diagnosed at a later age for NBDs [47 Autism Spectrum Disorder (ASD), 56 Developmental Coordination Disorder (DCD), 58 Attention Deficit Hyperactivity Disorder (ADHD)], were evaluated and compared to the records of 58 children with typical development. Ten covariates including 28 parameters were accumulated, compared and statistically analyzed.

Results: Deviation from trajectories of seven parameters (gestational age, birth weight, head circumference percentile, weight percentile, gross motor development, difficulties in communication and language) seem collectively to predict ASD with 85% probability. The first five parameters may collectively predict DCD with 72% probability. Predictors of ADHD with 58% probability have been described (Gurevitz et al 2014).

Conclusions:This cohort retrospective study illuminates risk factors at infancy that may predict the development of NBDs. Most prominent are deviations from weight followed by deviations in head circumference trajectories, and delay in motor development due to muscle strength and tone irregularities. Identification of risk factors at infancy is crucial for early intervention programs, such as prevention of rapid changes in weight and manipulations to strengthen motor development and the corresponding neural circuits. The sooner risk factors are recognized, more efficient an early tailored bio-psycho-social treatment might be.

166

Primary Care

KKI- NECT : A Neurodevelopmental and Mental Health Tele-Education Network for Paediatric Primary Care Clinicians

Mary Leppert ^1,2 , Joyce Harrison ^1,2 , Nancy Grace ^1,2

¹ Neurodevelopmental Medicine and/or Psychiatry/Behavioral Psychology, Kennedy Krieger Institute, USA

² Pediatrics and/or Psychiatry, Johns Hopkins University School of Medicine, USA

Background: There are growing concerns in the U.S. about the increasing prevalence of paediatric neurodevelopmental and mental health disorders, now 15% and 11-20% respectively. There is a critical shortage of board certified neurodevelopmental, developmental/behavioral or child psychiatry subspecialists to care for this vulnerable population. Per the American Board of Pediatrics’ estimates, there is one board certified subspecialist for every 100,000 U.S. children. Given workforce shortages, paediatric primary care clinicians (PPCCs) are increasingly tasked with identifying and managing neurodevelopmental and mental health disorders. However, surveys of practicing paediatricians indicate gaps in the training, knowledge, and confidence to meet this need.

Objectives: Approaches to preparing the PPCC workforce include high quality continued medical education (CME) and integrated care models. Through guided practice and mentorship, the Kennedy Krieger Institute Network for Early Childhood Tele-Education (KKI-NECT) aims to expand PPCC workforce capacity to identify and manage neurodevelopmental and mental health disorders within the medical home.

Method: KKI-NECT replicates the Project ECHO® hub-and-spoke model, offering CME instruction on neurodevelopmental and mental health disorders. Each virtual clinic combines a case presentation by a spoke member and didactic content aimed at guiding PPCC practice. Didactic presentations are based on a multidisciplinary, multilevel curriculum authored by KKI/Johns Hopkins faculty.

Results: A remarkable number of cases (69%) are complicated by co-occurring neurodevelopmental and mental health concerns. Further, 59% of children had at least one known Adverse Childhood Experience (ACE). With KKI-NECT guidance, nearly all cases have been managed within the Medical Home.

Conclusion: Based on KKI-NECT data, PPCCs are increasingly entrusted with caring for children with ACEs, neurodevelopmental and mental health disorders, and very often co-occurring conditions. Project ECHO® appears to be an effective strategy for guiding PPCC identification and management of neurodevelopmental and mental health disorders. Thus, KKI-NECT provides an effective solution to the paediatric subspecialty workforce shortage.

372

Primary Care

Experiences with Hypnotherapy in Primary Care Pediatric Practice

Helena Mole ¹

¹ Primary Care Pediatric Office, Pediatrija Helena Mole, Slovenia

Background: The last 50 years is the era of modern hypnotherapy in children. Thanks to mounting research work hypnotherapy is now succesful evidence-based method of treatment in many pediatric conditions.

Primary care pediatrician interested in the field can achieve proper education on hypnotherapy. In primary care pediatric practice there are many children that can profit if the help is offered on spot and in much less time than referral to the specialist.

Objective: In three years after finishing the national training program on hypnotherapy more than 150 children were offered help with hypnotherapy in my primary care pediatric office.

Methods: The results of the therapy were observed retrospectively. Regarding the cause for using hypnotherapy we observed the results of hypnotherapy in three groups – Nocturnal enuresis, Anxiety and fears and Tics.

Results: Nocturnal enuresis – 41 children were treated (age group 4,5 to 11,5 years, boys in 2/3), successfully in 28 (68%), on average they needed 3 sessions.

Anxiety and fears – 40 children were treated (age group 4,5 to 18 years, equal by gender), successfully in 35 (87%), on average they needed 2,5 sessions, 12 (34%) of successful cases needed only one visit.

Tics – 15 children were treated (age group 5,5 to 13 years, boys in 2/3), successfully in 12 (80%), on average they needed 2 sessions.

The children with other diagnoses were also treated with hypnotherapy but the numbers are too small to present the results. Chronic pains, sleep disorders and stuttering are some examples in which hypnotherapy can be very helpful.

Conclusion: Hypnotherapy can be a very helpful method in primary care pediatric practice.

164

Primary Care

Developing and Implementing Asthma Severity and Control Screening in a Large Urban Health System

Marina Reznik ¹ , Philip O. Ozuah ¹

¹ Pediatrics, Montefiore Medical Center, USA

Background: Asthma severity or level of control classification is the first step in asthma management. To optimize asthma management, screening must be done at each visit.

Objectives: 1) To develop and implement asthma severity and control screening within electronic health record (EHR) of a large urban health system; 2) To increase asthma screening from 0% to 80% by December 2020.

Methods: We developed an asthma screening algorithm in EHR based on national guidelines. Screening is conducted by the nursing staff prior to patient being seen by the provider. Based on responses, severity/control classification is calculated and displayed in EHR. Classic Quality Improvement tools such as the Model for Improvement and Plan Do Study Act (PDSA) cycles were used to incorporate changes into daily workflow. Practice Advisory Board meets monthly to discuss barriers to implementation and identify solutions. The Board makes decisions on adopting successful strategies and brainstorm new strategies to address barriers.

Results: This project is ongoing and preliminary results are available from 3 practices: small pediatric, large pediatric and family medicine practices. To date, a total of 4,057 screenings have been completed. Screening rates improved from 0% to 78.6% in a small pediatric practice, from 0% to 66.7% in a large pediatric practice, and from 0% to 38.7% in a family practice (See figures).

Challenges encountered include: competition with other nurse-completed screenings; screening questions are available in English; nursing staff shortages; nurses forgetting to conduct screening; and caregiver not knowing information about child’s asthma.

Conclusion: We developed an asthma screening algorithm within EHR, trained nursing staff and implemented patient screening at the time of clinic visit allowing providers take action on asthma management based on the screening result. Further collaborative efforts are needed to improve and sustain screening rates with the ultimate goal of improving patient clinical outcomes.

768

Primary Care

Pocus Aiding in The Diagnosis of Infantile Pompe Disease In Pediatric Practitioner Office: Based Setting

Adib Salim ¹

¹ ATS Bergamo, Italy

Background: Pompe disease is a rare genetic disorder that affects glycogen and lysosome storage secondary to a deficiency in the enzyme acid alfa glucosidase, with a spectrum of severity attributed to the differing amounts of enzyme deficiency.

Objective: Point-of-care ultrasound (POCUS) modality offer a means of access to early diagnosis and appropriate treatment of rare disorders to achieve better quality of life.

Methods: We report the case of incidental bedside echocardiographic finding of mild LV septal wall thickness in 3 months of age infant prompted further investigations led to early diagnosis of Pompe disease.

Conclusions: Recognizing earlier this disease by POCUS and initiating enzyme replacement therapy led to improve survival, cardiorespiratory function, growth and development milestones of the patient.

536

Primary Care

I Have Seen You Before - Frequent Users of the Pediatric Emergency Room

Maria Sao Pedro ¹ , Gonçalo Vale ¹ , Mariana Simões ¹ , Rita Parreira ¹ , Susana Correia ¹ , Cristina Didelet ¹

¹ Pediatrics, Centro Hospitalar Barreiro-Montijo, Portugal

Background: Patients’ overuse of emergency room (ER) services is a serious and growing health care problem in Portugal. Particularly in pediatric age, there is often misuse of resources with overburden of the ER with non-urgent conditions. Recurring visits are also a major concern.

Objective: Characterize the most recurring patients of a pediatric ER service.

Methods: Descriptive study, conducted in a suburban hospital in Portugal. Sample consisting of the 20 most frequent users of the pediatric ER per year, in the last 3 years. Data regarding to patient’s visits to the ER, medical history and socioeconomic status were collected from their clinical file. Software Microsoft Excel was used for statistical analysis.

Results: 60 patients, 26 female, with median age at the first ER visit of 11 months (IQR 2,04)[4 days-16 years old] and an average of 20 visits each per year (SD 5,97)[15-49]. Almost all continued to be and/or were frequent users of the ER in the previous years, with multiple recurrences during the same acute episode (48 out of 60), sometimes more than once per day (31 out of 60). A significant number of romanies was registered. Almost half the sample didn’t have chronic diseases and among the ones that had, 22 of the 31 had recurrent wheezing/asthma. Although 41 of the 60 attended hospital consultations, 16 of them often missed the appointments. A similar behavior was noted regarding primary care follow-up (15 out of 60). There was a high prevalence (34 out of 60) of patients coming from low income families.

Conclusion: Healthcare education programs, especially for at-risk populations, alongside with optimized links between primary and secondary care are needed. Particularly in chronic patients, better management of the disease with empowerment of the patient/caregiver and easier access to ambulatory revaluation should also help reduce ER overflow.

619

Respiratory

Management of Bronchiolitis in Portugal, 2000-2015: Do Guidelines Have an Impact?

Jose Fontoura-Matias ¹ , Diana Moreira-Sousa ² , Alberto Freitas ^3,4 , Inês Azevedo ^1,2,5

¹ Centro Materno-Pediátrico, Pediatrics Department, Centro Hospitalar São João, Portugal ² Department of Obstetrics-Gynecology and Pediatrics, Faculty of Medicine, University of Porto, Portugal ³ Department of Community Medicine, Information and Health Decision Sciences - MEDCIDS, Faculty of Medicine, University of Porto, Portugal ⁴ Center for Health Technology and Services Research (CINTESIS), Faculty of Medicine, University of Porto, Portugal ⁵ EpiUnit, Institute of Public Health, University of Porto, Portugal

Background: Several studies report an excessive use of diagnostic tests and procedures in bronchiolitis, not supported by guidelines.

Objectives: We aimed to evaluate medical interventions in children hospitalized with bronchiolitis in public Portuguese hospitals, from 2000 to 2015, to evaluate the impact of the national guideline and assess variations between regions.

Methods: Data was collected retrospectively from an administrative database that contains all hospitalizations in mainland public hospitals. Cases were selected using the ICD-9-CM codes 466.11 (bronchiolitis due to respiratory syncytial virus) and 466.19 (bronchiolitis due to other infectious organisms), in children under 2 years of age. For statistical analysis we used Chi-square test and logistic regression.

Results: In the 80,491 hospitalizations due to bronchiolitis, we found a high mean use rate of non-recommended diagnostic and treatmet procedures: chest x-ray (66.5%), blood analysis (56.5%) and respiratory secretions analysis (12.7%); nebulized therapy (83.5%), intravenous corticosteroids (24.2%), intravenous antibiotics (26.0%), electrolytes infusion (37.6%), and chest physiotherapy (20.4%). Over time, there was a gradual change in attitudes (Ptrend 0.001), with significant variation between regions. Centre region registered the lowest mean rates of routinely non-recommended procedures.

Conclusions: In this first national study, rates of the non-recommended diagnostic and treatment attitudes in bronchiolitis were higher than desirable, although there was a modest decrescent trend in their use over time. The observed variations were mainly dependent on the region, with no clear impact of the national guideline in attitude changing, highlighting the need for more active measures.

601

Rheumatology

Phenotypic and Genotypic Characteristics and Damage Accrual of Monogenic Autoinflammatory Diseases other than Familial Mediterranean Fever from the Pediatric Rheumatology Arab Group (PRAG)

Sulaiman Al-Mayouf ¹ , Sulaiman Al-Mayouf ¹ , Abdualziz Almutairi ¹ , Safia Albrawi ² , Abdulatif AlEnazi ⁸ , B Fatallah ⁴ , Abdullah Alsonbul ¹ , Moahmmed Abu-shukair ³ , Raed Alzyeoud ³ , Adel Alwahadneh ³ , Mubruka Zlenti ⁷ , Ebtisam Kawaja ⁷ , Khloud Khawaja ⁵ , Zakia Almusawi ⁶ , Wafa Madan ⁶ , Muna Almuatiri ¹⁰ , Nora Almuatiri ⁹

¹ Pedaitric Rheumatology, King Faisal Specialist Hospital & Research Center, Saudi Arabia ² Pedaitrics, Royal Hospital, Oman ³ Pedaitrics, Queen Rania Children Hospital, Jordan ⁴ Pedaitrics, Jalilah Children Hospital, United Arab Emirates ⁵ Pedaitrics, Mafraq Hospital, United Arab Emirates ⁶ Pedaitrics, Salmanyah Hospital, Bahrain ⁷ Pedaitrics, Tripoli Children Hospital, Libyan Arab ⁸ Pedaitrics, King Fahad Medical City, Saudi Arabia ⁹ Pedaitrics, AlSabah Hospital, Kuwait ¹⁰ Pedaitrcs, Adan Hospital, Kuwait

Objective: To report the phenotype, genotype and response to treatment in Arab children with monogenic autoinflammatory diseases (AIDs) other FMF, with focus on accrual damage.

Methods: We retrospectively reviewed patients with clinical and/ or genetically proven monogenic AIDs other than FMF seen between 1990 and 2018 at 10 rheumatology clinics from seven Arab countries. Data were collected at the last follow- up visit comprising consanguinity, age at onset and diagnosis, clinical and laboratory findings, treatment, damage accrual and death related to monogenic AIDs.

Results: Seventy (46 female) patients with monogenic AIDs were analyzed. Consanguinity rate was 74.6% and a family history of AIDs was present in 60%. The mean age at disease onset was 3.2±2 years. The initial diagnosis was inaccurate in 47% and the mean diagnosis delay was 4±3 years. The most frequent monogenic AIDs were LACC1 associated monogenic JIA and monogenic Crohn’s (23) followed by CAPS (12), TRAPS (12), HIDS (9), Majeed’s syndrome (6). Musculoskeletal involvement was the main feature in LACC1 associated monogenic disorders. Genetic testing was performed in 67 patients, 69% had genetically confirmed disease. Patients with mutation c.T850C p.C284R in exon 4 of LACC1 had severe arthritic changes. Three CAPS patients with NLRP3 mutation had cognitive impairment and one with significant hearing and ocular damage. Two HIDS patients had homozygous p.V3771 mutation and other two patients with p.V3771/compound heterozygous MEFV: p.E148Q/p.P369S/p.R408G. Three different LPIN2 mutations were recorded for Majeed’s syndrome. Overall, growth failure was the most frequent (36%), followed by cognitive impairment (13%). There were three deaths due to infection.

Conclusion: The number of genetically confirmed patients with monogenic AIDs other than FMF are not uncommon among Arab children probably due to a high consanguinity rate. Diagnostic delay and high damage accrual emphasize the need for more awareness and early referral to specialized centers.

EAP 2019 Congress and Master Course

Poster Presentations

1053

Adolescent Medicine

Young People in Crisis- Assessing Risk of Mental Health Patients in the Children`s Emergency Eeparment

Adla Alattas ¹ , Amit Gite ¹ , Kim Rundell ¹ , Kim Pierson ¹ , Elizabeth Bessell ¹ , Ana Merino ¹

Elia ¹

¹ Paediatrics, Lister Hospital, UK

Mental health (MH) disorders in children and young people (CYP) is on the rise with many CYP presenting acutely to the emergency department (CED) in ‘crisis’.CYP presenting to CED will be initially triaged and cared for by non-MH professionals while awaiting assessment by a children’s crisis team. There are often complex and multiple issues for these CYP in addition to their medical needs. A Mental Health Risk Assessment (MHRA) tool was introduced to help non-psychiatric trained medical staff in their initial and ongoing assessment.

To conduct a demographical analysis of CYP presenting to CED with acute MH issues in 2018 as well as evaluating our MHRA-tool in risk assessing these patients.

Methods: We collected data including age, gender, diagnosis and duration of admission for all CYP presenting to CED with acute MH problems over a one-year period. Following the introduction of a MHRA-tool we collected feedback from CED nurses and healthcare assistants over a 3-month period.

Results: There were 380 MH admissions to CED over a one-year period. Age of presentation was 9 -16 years of age. There was a female:male ratio of 2.7:1. 33% of CYP presented with self-harm. Nearly 40% of CYP presented with suicidal attempts or ideation. Over 80% were admitted for 24-hours, with the average wait for crisis team assessment being about 17 hours. Over 60% of nursing staff found the MHRA-tool a useful means to ask questions relating to potential risk in CYP during admission.

Conclusion: CYP with MH disorders who present to CED in ‘crisis’ often have multiple and complex issues.

Non-MH trained medical staff need a strategy and formal means to make a risk assessment while CYP await formal psychiatric review.

We recommend using a locally approved MHRA-tool to objectively risk stratify and assess such patients whilst waiting for a mental health assessment.

515

Adolescent Medicine

Pyometra: A Rare Cause of Acute Abdomen in Prepuberal Girls

Maria Bauluz-Barcena ¹ , Pilar Fernández-Eire ² , Laura González ³ , Irene Garcia ³ , Vanesa Rodriguez ³ , Emilio Couceiro ³

¹ Pediatrics, Hospital Alvaro Cunqueiro, Spain ² Pediatric Surgery, Hospital Alvaro Cunqueiro, Spain ³ Gynecology, Hospital Alvaro Cunqueiro, Spain

Background: Pyometra is the accumulation of purulent fluid in uterine cavity. It is a severe, life-threatening condition. This rare entity is mostly described in postmenopausal women with acquired stenoses of external cervical OS. However, some congenital malformations of female genital tract have been reported as an infrequent cause of pyometra.

Objective: To describe a rare entity with a challenging diagnosis.

Method: We report an exceptional case of a 13-year-old patient with generalized peritonitis due to pyometra. She had no previous medical records, neither had she reached menarche. She presented on Pediatric Emergency Department with severe suprapubic abdominal pain and anorexia for the last 4 days. As she would add later, she had been suffering from suprapubic pain, diarrhea and vomiting once in a month, for the last 6 months. Physical exploration: abdominal guarding, intense hypogastrium pain, normal female genitalia, low-grade fever. Analytical: leukocytosis, increased CRP. On suspicion of acute abdomen, an urgent abdominal ultrasound was made, showing fluid collection in uterine cavity that extended to peritoneal cavity through Fallopian tubes. Gynecologists, under general anaesthesia, performed ultrasound-guided introduction of a grooved probe, through a completely stenotic cervical OS into the uterine cavity, in order to drain the uterus (picture 1). Later, a pediatric surgeon performed a laparotomy, profuse lavage and evacuation of purulent fluid from peritoneum (picture 2). Reproductive organs were preserved, and a Foley baloon catheter was left inside the uterus for 2 weeks. No incidents were recorded during postoperative evolution, so she was discharged. One month later, she was asymptomatic and did not reach menarche yet.

Conclusions: Peritonitis is not always appendicular. Although treatment of pyometra in postmenopausal women is well established involving hysterectomy, it might be challenging in pubertal girls, as preservation of reproductive organs is a priority. Long-term effects over fertility in this patient remain still uncertain.

609

Adolescent Medicine

Art Based Support Sessions for Paediatric Inpatients in a District General Hospital

Shavindra Chellen ¹ , Yasmin Dosanjh ¹ , Su Laurent ¹ , Vaseem Hakeem ¹

¹ Children's Services, Royal Free London NHS Foundation Trust, UK

Background: In the United Kingdom, there is an increasing emphasis on supporting the additional needs of children and young people in hospitals with the use of schoolteachers and play therapists. Art based support sessions provide a dedicated way for children and young people to express their feelings non-verbally and develop a sense of achievement, positivity and self worth during their inpatient stay.

Objective: Art based support sessions create a sense of achievement and provide a purposeful activity for inpatients, increasing their resilience whilst also encouraging interaction. Personal expression can also help patients process personal issues, which in turn may help with mental health conditions. The positive relationships formed with an art therapist can also enable patients to develop better coping strategies. Art based support sessions induces ‘flow’ – which is defined as a subjective psychological state of total involvement in an activity. Often cited as bringing about highest sense of wellbeing and overcoming poor concentration.

Methods: Over a one-month period from May 2017 to June 2017, appropriate inpatients were identified by nursing and medical staff and offered the chance to attend an art based support session. Inpatients who volunteered attended one session with a student art therapist. Involvement in these sessions was on a voluntary basis and each session lasted for 1 hour.

Results: 10 patients were involved, ranging from 7 to 16 years old. Written feedback was collected from patients following each session. 70% (7/10) described the session as brilliant. 90% (9/10) wanted another session. 80% (8/10) would like the session to be longer.

Conclusion: Our feedback shows that children enjoyed these sessions and they distracted them from stressors they faced whilst being hospital inpatients. During these sessions we believe that children experienced a sense of achievement and positivity, developed increased resilience and had an opportunity for personal expression.

303

Adolescent Medicine

How well are we Involving Children and Young People (adolescents) in Consent to their Surgery? An Audit of Practice

Sherine Dewlett ¹ , Deborah Davidson ¹

¹ Paediatrics, Royal National Orthopoedic Hospital, UK

Background: It is good practice to involve children and young people (CYP) in consent to treatment. 16-17 year olds are presumed to have capacity. The Royal National Orthopaedic Hospital is a specialist centre performing complex often high risk orthopaedic surgery on CYP , a high proportion of which are adolescents and vulnerable children .

Objectives: We sought to find out how many children are involved in the consent process, if mental capacity was assessed and if parental responsibility was documented with a view to improve the quality of the consent process.

Methods: A retrospective audit of electronic notes of children admitted in August 2018.

Results: 108 patients assessed. 22 (20%) were 16-17 year olds , 78 (72%) were 10-17 year olds (adolescents) . 72% of 16-17 year olds countersigned forms but parents gave primary consent . Only 4% of 16-17 year olds gave primary consent with parents counter signing. Involvement increased with age : 50% of 10 year olds ; 80% of 17 year olds countersigning forms. Senior doctors gained informed consent- 70% being consultant or registrar level. Mental capacity assessment ( MCA) was not documented . Parental responsibility was assessed 94% of the time. Only 1 child may have lacked capacity; consent was signed by parents and MCA was not documented.

Conclusion: we are involving CYP in consent to surgery but can improve numbers signing consent forms and documentation of MCA. We have tackled this by educational interventions to doctors seeking consent ; a short film with adolescent involvement aimed at CYP and their families ; an opt out policy of consent rather than opt in by using form 1 (primary consent to treatment) in 16-17 year olds rather than form 2 (parental consent ) and added MCA documentation in the admissions booklet and will re-audit.

990

Adolescent Medicine

Shortage of Children and Adolescent Mental Health Service (CAMHs) Beds is the Most Common Reason in Delayed Transfers of Care (DTOCs) in Paediatric Ward

Lucy Walker ¹ , Sanjay Wazir ¹ , Mojgan Ezzati ¹

¹ Paediatric, Homerton University Hospital NHS Foundation Trust, UK

Background: There is a national shortage in CAMHS beds throughout the UK. Additionally, discharge pathway for adolescent and young children with safeguarding and mental health concerns in paediatric ward encompasses close working relationship between acute, mental health and social care services. Yet, there is increasing pressure on all services in regards to patient flow which compromise this partnership hence patient care.

Objectives: To facilitate discharges of paediatric patients with mental health and safeguarding concerns by counting and collecting data about delayed transfer of care from acute setting to mental health or social care services.

Methods: A retrospective survey was conveyed on all paediatric admissions with mental health and safeguarding concerns over a period of 12 months (March 2018-March 2019) at our paediatric ward at Homerton University Hospital. A total of 25 adolescent patients were admitted in the paediatric ward with mental health and safeguarding concerns during this period. Data were collected from their case notes about the safeguarding, medical and mental history and management. Also, data was collected to compare the dates when they were medically fit for discharge with when they were safe to be discharged including the reasons behind delay for discharge.

Results: There was a total of 57 days over the period of 1 year when patients were medically fit for discharge but not fit for discharge due to shortage of CAMHS beds, awaiting discharge planning meeting, court hearing and housing issues. Of note the most common reason in delay in transfer of care was due to shortage of CAMHS beds followed by housing problem. However, in just less than half admissions, there was no delay in transfer of care.

318

Adolescent Medicine

Intentional Drug Intoxication in Adolescents: Who, When, How and Why?

Ana Bernardo Ferreira ¹ , Sara Rodrigues ¹ , Diana Reis Monteiro ¹ , Joana Cardoso ¹ , Lúcia Gomes ¹

¹ Pediatric/Neonatology Department, Centro Hospital Entre Douro e Vouga, Portugal

Background: Self-harm behavior in adolescents is a worrying reality. Understanding the patterns and risk factors underlying Intentional Drug Intoxication (IDI) may help prevent it.

Objective: To characterize IDI cases with prescription drugs in adolescents, managed in the emergency department (ED) of a level II hospital, between 2014 and 2018.

Methods: Retrospective study of IDI in adolescents, ages 12 to 17. Through consultation of medical records, we analyzed patient sociodemographic, time of ingestion, type, number and owner of medication used, management in ED and at discharge, and psychopathology history.

Results: There were 90 episodes of IDI in 85 individuals (71 female). Mean age at first episode was 15 years (similar to median value). Oral intake was the only intoxication route; in 45 cases, only one drug was taken. The most used group of substances were psychiatric drugs (50,4%). In 36.6%, the drug belonged to the patient and was a psychotropic medication. Most episodes occurred during the afternoon and at night, and during spring and winter. The main motivation for drugs intake was family conflict (42,2%), followed by problems in romantic relationships (28,9%). Seventy patients had a psychiatric evaluation before discharge; 4 were admitted to a psychiatric service due to structured suicidal intent. Sixty-three percent had a previous history of psychiatric/psychologic follow-up. Psychopathology or history of addiction was present in 35.3%. In 27,1%, parents were divorced. Seven had a history of institutionalization, and other 16 cases were under social services following.

Conclusion: Immaturity, misjudgment, impulsivity and lack of coping strategies are related to self-harm behavior, including IDI, in adolescents. Most times, there is no apparent structured suicidal intent. Emotional conflicts, social problems and a personal and family history of psychopathology are risk factors for this behavior. It is important to implement preventive measures, as follow-up of risk patients in a pediatric/psychiatric consultation.

578

Adolescent Medicine

Internet Addiction, A New Epidemics?

Carmen-Raluca Ghionaru ^1,2 , Elena Ruja ^1,3 , Vlad-Alexandru Ghionaru ^4,5

¹ Family Medicine, Romanian Association for Pediatric Education in Family Medicine AREPMF, Romania

² Family Medicine, Family Medicine Practice, Romania

³ Family Medicine, Family Medicine Practice, Romania

⁴ Faculty of Law, University of Bucharest, Romania

⁵ Sorbonne Pantheone I, College Juridique franco-roumain d'études européenes, Romania

Background: Being concerned about internet addiction, American researchers have included this condition in compulsive-impulsive disorders. In some countries, officials have set up treatment centers or organized educational programs in schools to stop the spread of the epidemic. In 2016, the World Health Organization included game addiction in the International Diseases Index (ICD11), including in this category also computer game or online addiction. In Romania, we have no real data on the extent of this phenomenon or the need to implement educational programs in schools.

Aims and Objectives: Identify and quantify the forms of internet addiction among secondary school, high school and university students.

Material and Methods: We conducted a qualitative observational survey on a total of 404 subjects, who completed a standardized questionnaire that quantifies internet dependency and classifies it in three mild, moderate, severe forms.

Results and Discussions: 77.6% of the subjects are internet-dependent to a certain extent (64.7% mild dependence, 12.5% medium and 0.4% severe). We noticed that the proportion of those who have the medium form of internet addiction is increasing as they get older. (58% middle school, 67% secondary school and 74 students), while the rate of no addiction persons decreases with age. There were no forms of severe dependence among high school students.

Conclusions: The study shows that we may be on the verge of an internet abuse epidemic and the necessity to implement educational programs in schools with the goal of acquiring the ability to use the internet for useful purposes, without being at the risk of developing addiction.

488

Adolescent Medicine

New Trends of Substance Use in Teenagers

Ana Lia Goncalo ¹ , Mariana Maia ¹ , Ana Luisa Correia ¹ , Sara Silva ¹ , Ana Aguiar ¹

¹ Pediatrics, Unidade Local de Saúde Matosinhos - Hospital Pedro Hispano, Portugal

Background: Adolescence is a period of important physical, psychological and emotional development. The need to become independent and to experience new sensations is associated with risk behaviors, such as use and abuse of tobacco, alcohol and illicit drugs.

Objective and Methods: To evaluate new trends of substance use in teenagers, by searching medical databases and journals for published articles in the last 5 years, using the keywords “substance”, “use”, “adolescents”

Results: The most common substances used by adolescents are alcohol and tobacco, as they are more readily available. Although a decline of cigarette smoking in adolescents has been observed in the last decades, e-cigarettes are recently gaining popularity and their use has been associated with initiation and then transition to more frequent cigarette smoking. Despite the similar decrease in consumption among teens, alcohol remains the main cause of intoxications admitted to the Pediatric Emergency Department in several European countries (including Portugal) and the differences between genders’ consumption habits tend to disappear. Marijuana is increasingly displacing alcohol and cigarettes as the first substance used, and is causally associated with subsequent polysubstance use. Since the mid 2000s New Psychoactive Substances (NPS) have been available on the market, specifically designed to imitate the effects of established illicit drugs, and untruly known as legal. Their use among adolescents is still low, but increasing, as they reported being more likely to try drugs if they are legal.

Conclusion: Adolescent substance use has been declining worldwide. This decreasing trend is probably a result of intensively provided preventive and educational measures, increasing the public awareness of the severity of underage substance use. Of major concern, the more prominent role of marijuana in the early stages of drug use and the rise of NPS, given the considerable potential for harm, including hospitalizations, overdose and fatalities.

681

Adolescent Medicine

Post-Traumatic Panhypopituitarism in a Teenager- A Multidisciplinary Challenge

Mihaela Gutu-Robu ¹ , Raluca Maria Vlad ^1,2 , Irina Dijmarescu ^1,2 , Daniela Pacurar ^1,2

¹ Paediatrics, Grigore Alexandrescu Emergency Children's Hospital, Romania

² Paediatrics, Carol Davila University of Medicine and Pharmacy, Romania

Background: Post-traumatic panhypopituitarism in children is a rare plurisymptomatic complication of traumatic brain injury, with major impact on the child’s development and quality of life. Endocrine screening after trauma is controversial.

Objective: We highlight the steps leading to diagnosis in the case of an adolescent hospitalised for unrelated pathology in order to increase awareness on this rare condition among physicians.

Methods and results: We report the case a 17-year-old boy admitted in March 2019 in „Grigore Alexandrescu” Hospital, Bucharest. The adolescent had a history of severe brain injury (age 13) due to a car accident. He was hospitalized 4 years later in the Orthopaedics Department for surgical correction of right hip chronic epiphysiolysis. The laboratory tests showed liver cytolysis, so he was transferred to the Paediatrics Department. The physical examination revealed coarse features, dry, harsh skin, coarse hair, the absence of face and body pilosity, absent secondary sex characteristics, infantile genitalia, hypopigmented skin lesions, inferior limb inequality with no other pathologic findings. Laboratory investigations showed decreasing transaminases, positive serology for EBV, low values of thyroid hormones, inadequate TSH, hypocortisolaemia, low testosterone and IGF 1. Ophthalmologic evaluation revealed post-traumatic right eye cecity. The endocrinology consult set the diagnosis of panhypopituitarism. Substitution treatment was recommended (Prednisone, L-Thyroxine, Testosterone). The cranio-cerebral MRI showed small pituitary gland, right deviated pituitary stalk, unidentifiable neurohypophysis and right optic nerve, parasphenoidal herniation of the arachnoid. Hip surgery was postponed due to potential anesthesia risks. He was referred to the Endocrinology Department for further assessment and treatment.

Conclusion: Post-traumatic rare complications such as panhypopituitarism should be considered even a long time after trauma. Focusing on solving the trauma might lead to a late diagnosis of the condition. A well-established multidisciplinary approach is needed if the child is to safely overcome any acute condition, considering the severe cortisol deficiency.

703

Adolescent Medicine

Risk Assessment of Liver Pathology in the Treatment of Juvenile Idiopathic Arthritis in Adolescence

Olga Pavlova ¹

¹ Department of Adolescent Medicine, Kharkiv Medical Academy of Postgraduate Education, Ukraine

Background: Juvenile idiopathic arthritis (JIA) is the most common type of arthritis in children under the age of 16. Liver disturbance has been attributed not only to the liver disease associated with rheumatoid diseases themselves but also with such factors as fatty infiltration, drug toxicity, thrombotic accidents, or autoimmune liver disease. AST to Platelet Ratio Index (APRI) is relevant for determining the cohort of children that requires further liver examination.

Objective: To study the most vulnerable contingent of patients with liver damage in the treatment of JIA methotrexate using the APRI index

Methods: A total of 73 children aged 10 to 18 years old, 43 girls and 30 boys were observed. The children were divided into five groups by treatment with methotrexate (MTX) (I - did not receive MTX (15 children), II - received up to 1 year (16 children), III - 1-3 years (13 children), IV - 3 -6 years (14 children); V - more than 6 years (15 children)). The APRI index was based on the generally accepted formula.

Results: In children with JIA, who did not receive methotrexate APRI index was 0.34 ± 0.04. Patients in group II - 0.61 ± 0.18 (p

Conclusion: Children receiving methotrexate up to 1 year have highest risk of liver damage. Use of methotrexate more than 1 year reduces risk of liver disease. For a clearer understanding of pathological changes in the liver, further research is needed.

485

Adolescent Medicine

Clustering of Health-Related Behaviors and its Relationship with Individual and Context Factors in Portuguese Adolescents: Results from a Cross-Sectional Study

Constanca Santos ^1,2 , João Picoito ^1,3 , Isabel Loureiro ¹ , Carla Nunes ¹

¹ Escola Nacional de Saúde Pública, Universidade NOVA de Lisboa, Portugal

² Department of Pediatrics, Centro Hospitalar Cova da Beira, Portugal

³ Department of Child and Adolescent Psychiatry, Centro Hospitalar Universitário de Coimbra, Portugal

Background: Health behaviours are shaped early in life, laying the foundations of future health. Unhealthy behaviours tend to occur in complex specific patterns and are influenced by individual and context factors.

Objective: We aimed to identify and characterize patterns of health-related behaviours among Portuguese adolescents and correlate them with individual and context factors.

Methods: This study was based in Portuguese 2009/10 survey of Health Beahviour in School Aged Children Study, comprising 4036 adolescents. Individuals were partitioned into groups using two step cluster analysis based on 12 variables regarding diet, physical activity, screen use and substance use. The association between cluster membership and individual, family, school and peer factors was calculated based on crude and adjusted odds ratio using a multinominal regression.

Results: The median age was 13,6 and 54% were of the female gender. Overweight and obesity were highly prevalent (25%), as of sedentarism, with only 13% exercising daily. 78% lived with both parents and 59% had medium-low affluent families. We identified four distinct behavioural clusters: “Active screen users” (31,1%), “Substance users” (13,3%), “Healthy” (26,4%) and “Sedentary low fruit and vegetable eaters” (29,1%). Sociodemographics varied across clusters, showing a positive and significant association between male gender, poor family communication, violent behaviours, poor academic performance and school attachment and the “Substance users” and “Active screen users” clusters and between a low socioeconomic status and the “Sendentary low fruit and vegetable eaters” cluster. We found no association between nutritional status and cluster membership.

Conclusion: Cluster analysis identified several health compromising behaviour patterns. The understanding of these behavioural clusters and its relationship with individual and context factors is of extreme use to Public Health, allowing tailored health-promoting interventions. Further research is needed to understand how cluster membership evolves over time and its influence on nutritional status.

734

Adolescent Medicine

Adolescents in the Emergency Room –Three Years in a Pediatric Medical-Surgical Emergency Department

Joana Santos ¹ , Silvia Almeida ¹ , Inês Sobreira ¹

¹ Pediatric Department, Centro Hospitalar do Baixo Vouga, EPE, Portugal

Background: World Health Organization defines adolescence as the group from 10 to 19 years old. This stage is characterized by fast physical, psychological and social changes. Despite being overall healthy, high prevalence of risk behaviors rises morbidity in this group. It is important to recognize the leading causes of adolescent admission in the emergency services, to invest in prevention, early intervention and promote health.

Objective: Characterize adolescent admissions in emergency department.

Methods: Retrospective analysis of informatic records of adolescent emergency episodes, observed from January, 2014 to December, 2016, at the Pediatric Emergency Department of Centro Hospitalar do Baixo Vouga, EPE.

Results: In the analyzed period, there were registered 38363 emergency adolescents episodes. 67% of the admitions were due to “DISEASE”, 24,1% due to “ACCIDENTS” (road, sports, personal, work, domestic and school accidents), 0,7% were “INTOXICATIONS”, 0,5% for “ASSAULT” episodes and 0,7% admissions of “PREGNANT AND PARTURIENT”.

One third of the “DISEASE” admissions were due to respiratory disease and one sixth due to digestive disease. Mental health and behavioral issues were responsible for 4% oh these diagnostics.

58,3% of the adolescents was observed only by Pediatrics specialists, and the others needed collaboration from other specialties.

Conclusion: 26% of the admissions were due to preventable causes, like accidents, intoxications, agressions or pregnancy. It’s urgent to act at primary prevention level, but it’s also important to take every contact chances to identify those at risk and decrease this group morbidity.

489

Adolescent Medicine

Health Care Improvement Facing Child Abuse. Mistakes to be Avoided and New Challenges to be Addressed

Eva Maria Navarro Villalba ² , Mariano Silva Hernandez ¹ , Virginia Santana ¹

¹ Pediatría, Hospital Universitario La Paz, Spain

² Pediatría, Centro de Salud Barrio del Pilar, Spain

Background: Child abuse is an increasing reason of consultation, either in Pediatric Primary Care (PPC) or in Pediatric Emergency Departments (PED). Healthcare professionals typically have not received proper training on the subject at hand. This results in mistakes in patient care that could be avoided.

Clinical case: We herein describe a study case of a 15-year-old male patient who is brought by his mother to our PED, referred from a tertiary-level Hospital after having conducted higher risk intercourse with an adult male. The youngster had arranged a meeting using a mobile phone application. At the first PED he visited, no clinical samples were collected, although medical examination was executed. Instead, the patient was directly referred to the National Police, who took judicial samples with the collaboration of a forensic doctor.At our PED, which he was later referred to, the patient and his mother handed the medical report from the first PED, so no new interrogations or physical examinations were carried out. Following our Hospital’s Child Molestation Protocol a blood and urine sample was tested for sexually transmitted diseases and a negative urine toxicity study was carried out. After evaluating the results, the patient began taking the post-exposure prophylaxis and antibiotic prophylaxis medicines. Finally, he was given an appointment with the Pediatric Infectology Consultation so that a proper follow-up could be carried out.

Conclusion: Child abuse is a frequent reason of consultation in PPC services, for which Healthcare professionals (HP) are not completely prepared to assist. Usually, they have not received proper training to do so, and they are not aware of the procedure to be followed in terms of observation, notification and management. Accordingly, a standardized Health Care Protocol should be developed and introduced. HP should receive specific and proper training to successfully address this issue and provide the best patient care possible. The need for the abovementioned is particularly strong, considering the increasing amount of cases taking place,and the new ones arising from the use of the New Technologies.

253

Allergy & Immunology

Improving of the Detection of Primary Immunodeficiency Diseases through Implementation of Educational Programs

Oksana Boyarchuk ¹ , Tetyana Hariyan ¹ , Maria Kinash ¹ , Lyubov Volyanska ¹ , Olga Rusanovska ² , Tetyana Kovalchuk ¹

¹ Department of Children's Diseases and Pediatric Surgery, I.Horbachevsky Ternopil State Medical University, Ukraine ² Department of Maternity and Childhood Protection, Uzhhorod National University, Ukraine

Background: Early detection of primary immunodeficiency diseases (PID) is vital for adequate prevention and management of PID infectious complications.

Objective of this study was to improve the primary immunodeficiency diagnostics through implementation of the model combining physician education and public awareness with the infrastructure to diagnose these diseases.

Methods: Three strategies combination has been followed from February 2017 in Ternopil region, Ukraine. The first was education of primary-care physicians (pediatricians, GP doctors) and other specialists (rheumatologists, otolaryngologists, pulmonologists, surgeons, etc.) in early PID symptoms and detection using workshops, trainings, and targeted publications. The second was organization of public events, media appearance to raise PID awareness. The third was undertaken immunological testing for patients with suspected PID.

Results: Workshops and trainings for primary care physicians on early PID detection were organized in 2017 in every regional district (15 districts were totally covered). Generally 540 physicians and nurses attended the lectures and workshops, including pediatricians, general practitioners, etc. We also visited the outpatient departments, talking to doctors, patients and their parents about the PID warning signs.

The examination of the children at risk for PID was conducted during the visits. We developed examination cards of children with suspected PID. These children were referred to the regional children’s hospital for further examination.

We have translated into Ukrainian and published educational materials: warning signs, testing stages. We have also created some educational materials about PID which were disseminated among physicians.

By the beginning of 2017, there were 11 children with PID in our region, at the end of 2018 their number increased to 31. We also follow 12 patients with suspected PID that need monitoring and examinations to finalize the diagnosis.

Conclusion: Implementation of the model combining physician education and public awareness with the infrastructure to diagnose these diseases is effective strategy to increase PID diagnosis.

799

Allergy & Immunology

Multimorbidity of Upper Respiratory Tract Pathology in Children with Bronchial Asthma

Tatyana Eliseeva ¹ , Svetlana Krasilnikova ² , Andrei Shakhov ² , Ivan Balabolkin ³ , Elena Tush ¹ , Vilya Bulgakova ³ , Olga Khaletskaya ¹

¹ Department of Pediatrics, Privolzhsky Research Medical University, Russia ² Department of Otolaryngology, Privolzhsky Research Medical University, Russia ³ Institute of Pediatrics, National Medical Research Center of Children's Health, Russia

Background: Bronchial asthma (BA) is a systemic allergic disease and is associated with upper respiratory tract (URT) pathology. In recent years, attention was focused on allergic diseases multimorbidity, while the spectrum of URT pathology in children with BA is not characterized enough.

Purpose: To study the structure of URT pathology in children with atopic BA.

Method: 358 children with atopic BA were examined, the average age of children was 9,91 (9,47; 10,35) years, of which 67,9% were boys (192/358), and 108 children with nasal breathing disorders, comparable in age and sex, but without BA. In addition to the standard all – clinical, allergological, functional examination, all patients underwent video endoscopic examination of the nasal cavity and nasopharynx.

Results: At children with BA were diagnosed with allergic rhinitis (AR) and/ or allergic rhinosinusitis (ARS) with a predominance of persistent forms. The «isolated» course of AR/ARS occurred in 11,7% (42/358) of patients; other children had nasal symptoms due to a combination of AR/ARS with other variants of URT pathology. Pharyngeal tonsilhypertrophy occurred in 61,2% (219/358) of patients, hypertrophic rhinitis - in 9,2% (33/358) of children with BA, nasal architectonics disorders were diagnosed in 50% (179/358) of patients. The combination of two nosological variants of nose pathology occurred in 47,8% (117/358) of patients with BA; 40,5% (145/358) of children with BA had multimorbidity of the nasal pathology – a combination of three or more nosological units.

Conclusion: For children with atopic BA and nasal symptoms often have combined and multimorbid forms of URT pathology. Verification of nasal obstruction causes allows to individualize therapy for patients with BA and minimize negative impact of URT pathology on BA course.

804

Allergy & Immunology

The State of the Autonomic Nervous System in Children with Bronchial Asthma

Elena Tush ¹ , Tatyana Eliseeva ¹ , Olga Khaletskaya ¹ , Vilya Bulgakova ² , Ivan Balabolkin ²

¹ Department of Pediatrics, Privolzhsky Research Medical University, Russia

² Institute of Pediatrics, National Medical Research Center of Children's Health, Russia

Background: Violation of vegetative regulation (VR) is a component of the pathogenesis of bronchial asthma (BA).

Purpose: Determine the relationship of VR parameters with the parameters of asthma control level in children using methods available in a wide clinical practice.

Method: 88 patients (54 boys and 34 girls) aged 5 to 17 years with atopic bronchial asthma were examined. Quantitative assessment of bronchial asthma control was carried out using questionnaires Asthma Control Questionnaire-5 (ACQ-5), Childhood Asthma control test (ACT-C) in children under 12 years old, and Asthma control test (ACT) in children and adolescents aged 12 years and older. All children underwent a standard examination with determination of blood pressure, pulse, respiratory rate, with the calculation of Kerdo and Hildebrandt indices, characterizing vegetative regulation. Taking into account the age-dependent changes in heart rate, we used for the first time a relative heart rate index equal to the ratio of the patient`s heart rate to the median heart rate for this age group.

Results: Correlation with ACQ-5 was obtained for the Hildebrandt index (r=0,45 p=0,0003), the respiratory rate (r=-0,27, p=0,032) and the relative pulse index (r=0.40, p=0,0012). The association of Kerdo index with ACT-C test values (r=-0,32, p=0,045) was established. In the group of patients with no BA control the Hildebrandt index was statistically significantly higher than in patients with control of the disease. In children with uncontrolled asthma it was 5,23±0,25 units that exceeds normal values and may reflect a mismatch in the work of cardiovascular and respiratory systems.

Conclusion: The interrelation of changes of vegetative regulation and the level of control of bronchial asthma in children is established, as well as the mismatch of the functioning of the cardiovascular and respiratory systems in children with uncontrolled asthma.

806

Allergy & Immunology

The Value of Thyroid Status for the Control of Bronchial Asthma in Children

Tatyana Eliseeva ¹ , Elena Tush ¹ , Elena Bolshova ¹ , Stanislav Ignatov ² , Vilya Bulgakova ³ , Ivan Balabolkin ³

¹ Department of Pediatrics, Privolzhsky Research Medical University, Russia ² Department of Chemistry, Lobachevsky State University, Russia ³ Institute of Pediatrics, National Medical Research Center of Children's Health, Russia

Background: Despite numerous studies, there was no consensus about the effect of thyroid hormones on asthma.

Purpose: To assess the relationship between the patient`s thyroid status and the level of BA control.

Method: 51 children with BA were examined, of them 15 girls aged 105.1+/-35.3 and 36 boys aged 114.2+/-14.1 months. All children underwent general clinical examination, spirometry, ACQ5 assessment, determination of thyroid-stimulating hormone (THS) levels, free thyroxine (free T4) test, and levels of antibodies to thyroperoxidase (anti-TPO) by ELISA.

Results: Elevated TSH levels occurred in 12 (23%) patients, while all of them had no clinical symptoms characteristic of hypothyroidism, and the maximum TSH rise was 8.45 mIU/L, which made it possible to treat this condition as subclinical hypothyroidism (cGT). None of the patients had an increase in the level of anti-TPO. Another 15 people had a TSH level in the range of 1.16-1.97 mIU/l, the remaining 24 had 2.01–3.74. 18 - had an excess of T4 free, but it was insignificant - a maximum of 23.1 pmol/l at the upper limit of the norm of 22 pmol/l, and was not accompanied by symptoms of thyrotoxicosis. Most often (in 33% of cases) subclinical hypothyroidism occurred in patients with complete control of BA (28, TSH 3.39±1.96), and was completely absent in patients with uncontrolled disease progression (5, TSH 1,87±0.73). In patients with partial BA control, TSH levels of 2.0 to 4.1 IU/L (18, TSH of 2.93±1.5) prevailed, which may indirectly indicate iodine deficiency. These differences do not reach the level of statistical significance (F =1.74, p=0.1858). Free T4 levels were comparable in all groups (16.57±1.93, 16.67±3.04, 15.82±4.93, respectively).

Conclusion: Patients with achieved BA control have a rather high incidence of subclinical hypothyroidism (23%).

808

Allergy & Immunology

Acute Allergic Reactions to Drugs in Pediatric Practice

Tatyana Eliseeva ¹ , Elena Tush ¹ , Polina Trushanina ¹ , Vilya Bulgakova ² , Ivan Balabolkin ² , Olga Khaletskaya ¹ , Stanislav Ignatov ³ , Elena Kareva ^4,5

¹ Department of Pediatrics, Privolzhsky Research Medical University, Russia

² Institute of Pediatrics, National Medical Research Center of Children's Health, Russia

³ Department of Chemistry, Lobachevsky State University, Russia

⁴ Department of Pharmacology, Pirogov Russian National Research Medical University, Russia

⁵ Department of Pharmacology, I.M. Sechenov First Moscow State Medical University, Russia

Aim: Assessment of the clinical and epidemiological picture of acute allergic reactions to drugs in children.

Methods: A retrospective analysis of the medical documents of 76 patients aged 3-17 years hospitalized in a children`s hospital due to acute hypersensitivity reactions to drugs was carried out.

Results: The average age of patients was 7.6 years [7.0-8.2]: boys - 34, girls - 42. In most children, cutaneous manifestations of hypersensitivity prevailed. Hypersensitive reactions to drugs of the type of acute urticaria are diagnosed in 21/76 (27.6%). In 17/76 (22.4%) children there was a combined development of urticaria rash and angioedema. All clinical manifestations of acute urticaria occurred in 38/76 (50%) patients. Various skin rash variants, including spotted-papular and morbiliform exanthema, which were quite rigid to therapy, in some cases prone to pigmentation, occurred in 31/76 (40.8%) patients, while 19 of them had a combination data of eruptions with symptoms of angioedema. In total, angioedema combined with cutaneous manifestations of hypersensitivity occurred in 36/76 (47.4%) patients hospitalized for drug hypersensitivity reactions. As the etiological causes of hypersensitive reactions to drugs in the sample of children, antibacterial drugs prevailed - in 45 of 76. In 12 children hypersensitivity reaction developed with the use of cephalosporins, 25 - aminopenicillin or protected aminopenicillin, in the remaining children - after the administration of cotrimoxazole, metronidazole, nitrofurans.

Conclusion: The most common clinical manifestations of side effects on medicines in children are urticaria and morbiiform (coripiform) exanthema. Attention is drawn to the high proportion of Quincke edema in this category of children, which can be very specific for children of childhood.

440

Allergy & Immunology

Are Our Children with Asthma and/or Allergic Rhinitis Well Controlled? A Study using CARATKids.

Ana Rita Constante ¹ , Zulmira Abdula ¹ , Susana Alexandre ¹ , Filomena Rebelo ¹

¹ Pediatrics Department, Centro Hospitalar Oeste - Caldas da Rainha, Portugal

Introduction: Around 12.9% of children and 11.8% of adolescents suffer from asthma in Portugal, 60-80% of which have allergic rhinitis concomitantly (ARA). Clinical control evaluation is essential and there is only one validated questionnaire for Portuguese children – “Control of Allergic Rhinitis and Asthma Test for Children” (CARATKids). It consists of 8 questions for the child and 5 for their parents, with a maximum score of 13. The following cut-offs were established: ≤3: controlled disease; ≥6: uncontrolled disease.

Objective: Evaluate clinical control of ARA in a group of children attending Paediatric Allergology consults using CARATKids and study the correlation between clinical score and peak expiratory flow (PEF), allergies in skin prick tests (SPT) and medication adherence.

Methods: Observational, descriptive and transversal study, using CARATKids in children diagnosed with asthma and/or allergic rhinitis, data from SPT, PEF and medication adherence. Statistical analysis with SPSS 23.

Results: We studied 31 children, mean age of 9.3 years (min. 6, max. 12). 93% had a correct inhalator technique and 38.7% an adequate PEF. 14.8% did not follow prescribed medication. The most common sensitisations in SPT were to mites. Regarding clinical scores, 38.7% had controlled disease and 22.6% uncontrolled disease, with a mean score of 3.97. The parents’ score correlated better to the final score than the children’s (p= 0,005 vs p=0,024). Lower respiratory symptoms were statistically correlated to clinical score (p=0,016). There was no correlation between PEF or medication adherence and clinical score. Positive SPT to Gramineae and Parietaria were associated with worse clinical scores (p=0,048 e p=0,05 respectively).

Conclusion: There is a tendency to undermine asthma’s and rhinitis’ symptoms, which may explain why only one third of the patients have controlled disease. Objective tools for clinical evaluation should be used frequently, such as CARATKids, to improve therapeutic adjustments and increase clinical control.

934

Allergy & Immunology

Child with Insect Sting Induced Anaphylaxis

Marija Dimitrovska-Ivanova ^1,2 , Irena Nikolova ¹

¹ Department of Pediatrics, Clinical Hospital Shtip, Macedonia

² Department of Pediatrics, Faculty of medical sciences, University "Goce Delcev" Shtip, Macedonia

Background: Anaphylaxis is an acute-onset and potentially life-threatening allergic reaction that can be caused by numerous allergic triggers. Children with asthma are at higher risk to develop anaphylaxis caused from medicines, foods, insect stings and bites.

Objective: To present a case of insect sting induced anaphylaxis in 6 years old boy with asthma history.

Methods: Six years old boy with asthma history admitted to the hospital due to severe bronchial obstruction. At admission pale, subfebrile (37,4C), with tachydispnea and weezing, using abdominal muscules, intercostal and jugular retraction. Barrel shaped chest and increased anteroposterior diameter. Auscultation-vesicular breathing with prolonged expirium and weezing. O2 sat=85%, WBC=20x10⁹/l, CRP=20mg/ml. Child was put on oxygen mask, inhalation with salbutamol, parenteralal corticotherapy and antibiotic. Continued regular inhalation maintenance therapy. On the fifth day, 15 minutes after parenteral application of Ceftriaxone child once felt the stinging pain in his right foot after it occurred aphonia, severe bronchial obstruction, swelling and redness of the face, lips, tongue, eyelids, ears and appearance of hives on the trunk and legs. Blood pressure = 90/50 mmHg, heart rate = 100/min, respiration rate = 40/min. Treated with oxygen mask, Amp. Adrenalin(1:10000) subcutaneous, anthistamines and corticosteroids intravenous, inhalation therapy with Adrenalin(1:10000), perenteral rehydration.

Results: After few hours with significantly reduction of redness and swelling on the face, lips, ears and eyelids, without hives on the trunk and legs and with easy bronchial obstruction treated inhalatory with salbutamol. Near the dorsum of the right foot was noticed insect sting with size of 2 mm. After two days child was discharged in stable condition without respiratory or other allergy symptoms.

Conclusion: Children with allergic background are at higher risk of developing anaphylaxis from various allergens, including insect stings. Those who have experienced anaphylaxis need to be provided and trained how to use auto-injectable epinephrine.

1000

Allergy & Immunology

Epidemiological Characteristics of Allergic Rhinitin in Children in Ukraine

Lyudmyla Duda ¹ , Olena Okhotnikova ¹ , Olena Sharikadze ¹ , Olena Usova ¹ , Nataliya Yakovleva ¹ , Tatyana Tkachova ¹ , Olena Ponochevna ¹ , Olena Oshlianska ¹

¹ Department of Pediatrics #1, Shupyk National Medical Academy of Postgraduate Education, Ukraine

Background. The results of recent studies have shown an increase in the prevalence of allergic rhinitis (AR) worldwide. Epidemiological information on AR in childhood proposes new hypotheses for further alethiological research into the genetic, lifestyle, environmental, clinical and medical-care factors for this disease.

Objective. To determine the prevalence of rhinitis in children and examine the age-old features of AR in Ukraine on the example of Kyiv region.

Methods. Written questionnaires (methodology by ISAAC) were self-completed at school by 13-17-year olds and completed at home by parents of children of younger age groups. 7106 children: group I – 1787 (6 month-5 years), group II – 2080 (6-7 years), group III – 1909 (13-14 years), group IV – 1330 (15-17 years). The gender ratio of the sample is about 1:1.

Results. The determination of the rhinitis symptoms prevalence was performed by assessment of the answers to question “symptoms of rhinitis in the past 12 months”, selected as the most sensitive criterion. The prevalence of rhinitis symptoms was 24,7% (759/7106), without significant gender difference (χ²=0,6509; p=0,419). In groups: I – 20,0% [95% CI: 18,2-22,0], II – 23,8% [95% CI: 21,9-25,6], III – 26,9% [95% CI: 24,9-28,9], IV – 29,6% [95% CI: 27,2-32,2]. The increase of rhinitis prevalence with age is observed (χ²=44,039; p=0). The prevalence of symptoms of rhinoconjunctivitis in groups: I – 10,6% [95% CI: 9,2-12,2], II – 11,3% [95% CI: 10,0-12,7], III – 14,8% [95% CI: 13,3-16,5], IV – 10,8% [95% CI: 9,2-12,6]. AR was diagnosed in 14,2% [95% CI: 12,6-15,9] in Group I, 19,3% [95% CI: 17,7-21,1] in Group II, 20,5% [95% CI: 18,7-22,4] in Group III , IV – 20,4% [95% CI: 18,2-22,6] (χ²=31,318; p=0).

Conclusion. The level of prevalence of AR in children of Ukraine in Kyiv region is quite high, which requires further study.

196

Allergy & Immunology

Fatal Streptococcus Pneumoniae Meningoencephalitis in a Fully Vaccinated 4 Year Old Boy

Roberta- Celine Ellul ¹ , David Pace ¹ , Simon Attard Montalto ¹ , Kristian Micallef ² , Andre Gatt ²

¹ Department of Paediatrics and Child Health, Mater Dei Hospital, Malta ² Department of Radiology, Mater Dei Hospital, Malta

A previously healthy four year old boy, born to non-consanguineous parents, had minor coryzal symptoms, fever and vomiting for 36 hours, treated empirically with oral antibiotics by GP. He was found jerking and unresponsive and rushed to hospital with a temperature of 37.8^OC and status epilepticus refractory to mannitol and repeated doses of midazolam and phenytoin. Shortly after admission, his conscious level deteriorated, requiring intubation and transfer to Intensive Care.

The white cell count was 44x10⁹ cells/L, with a neutrophilia of 41.31x10⁹cells/L. His CRP was 233mg/L on admission and 627mg/L after 2 days. A CT and MRI brain scans done within hours of admission revealed meningoencephalitis with widespread meningeal pus-infiltrates, cerebral swelling, impaired diffusion, raised intracranial pressure and brainstem herniation ( Refer to Figure 1 and 2). The intracranial bolt pressure was 91mmHg. Serum was PCR positive for Streptococcus pneumoniae, later confirmed as capsulated serotype 19F. He received ceftriaxone, vancomycin, metronidazole, clarithromycin and acyclovir without clinical improvement.Repeated brainstem function was absent after 3 days and life support was discontinued.

He had received all childhood vaccinations including four doses of a pneumococcal conjugate vaccine. There was no asplenia however immunoglobulins were low: IgG 1.2g/L (5.46-17.6), IgM 0.13g/L (0.26-1.21), and IgA 0.23g/L (0.52-2.84). Despite MMR and hepatitis B vaccination, he tested negative for Rubella and Hepatitis B surface IgG antibodies. A diagnosis of agammaglobulinaemia, possibly X-linked (XLA), was considered, particularly since a maternal first cousin had died of severe sepsis in childhood. In fact, subsequent DNA studies showed X-linked agammaglobulinaemia:c.871GC hemizygote in BTK gene. Maternal DNA is still being investigated.

The primary antibody deficiency syndromes, including XLA, usually present with recurrent respiratory tract infections due to an inability to mount an effective immunoglobulin response. Limited awareness of the presenting features commonly gives rise to diagnostic delays or fulminant septicaemia. Therapy consists of Ig replacement therapy and prophylactic antibiotics with resultant increased life expectancy by reducing the frequency and severity of infections. Earlier diagnosis with neonatal screening for those with a family history is the current ‘best option’, whilst bone marrow transplant and gene therapy offer a potential cure in these patients.

985

Allergy & Immunology

Pediatric Asthma: Knowledge of a School Community

Ana Esteireiro ¹ , Maria Gracinda Junqueira ²

¹ Pediatrics Department, Hospital de Caldas da Rainha, Centro Hospitalar do Oeste, Portugal

² Department of Public Health, ACES Pinhal Litoral, Portugal

Background: Asthma is a chronic disease frequent in pediatric patients. The school should have a leading role helping asthmatic students cope with the disease.

Objective: Characterize the knowledge of the school community Gualdim Pais about pediatric asthma.

Methods: Descriptive and transversal study, with application of an anonymous query. The frequency analysis was executed under Microsoft Office Excel®.

Results: 46 participants were included in this study, most were teachers and assistants (56.5% and 30.4%), with an average age of 44.3 years. Almost all (93.48%) recognize that asthma is common in pediatric age, 41.3% thinks this is an infectious disease and 24.9% reject that laughing can cause asthma crisis. Most of them (97.8%) know that asthma causes reversible airway obstruction and recognizes continuous care are required (76.1%). However, 41.3% of respondents believe that this disease is curable and 45.7% report being a rare cause of hospitalization. A minority (6.5%) believe that the practice of physical exercise is contraindicated in asthmatics. As for treatment, 87.0% know that includes medication control and crisis and 33.0% believes that inhaled therapy is responsible for gaining weight. In the opinion of 80.4% of individuals preventive measures may be implemented in schools which aim to prevent exacerbations in asthmatics.

Conclusion: Despite the therapeutic evolution, the asthma control is a challenge and this pathology is a frequent cause of hospitalization. The results express many doubts and myths about asthma so the focus on training is essential, taking into account the time spent by the child/adolescent in school. The training allows you to establish preventive measures and act correctly in case of crisis, promoting a better quality of life for asthmatics. Optimizing communication and coordination between health care, family and school promotes the improvement of care and reduction of hospitalizations.

983

Allergy & Immunology

Physical Development of Children with Nijmegen Syndrome

Tetyana Hariyan ¹ , Oksana Boyarchuk ¹ , Lyubov Dmytrash ³ , Svitlana Nykytyuk ²

¹ Department of Children's Diseases and Pediatric Surgery, I. Horbachevsky Ternopil National Medical University, Ukraine ² Pediatrics Department №2, I. Horbachevsky Ternopil National Medical University, Ukraine ³ Ternopil Regional Children Hospital, Ukraine

Background: Physical development (FD) is one of the indicators of child’s health in general and early diagnosis of primary immunodeficiencies.

Nijmegen Syndrome is a combined primary immunodeficiency represented by chromosomal instability and characteristic clinical manifestations: microcephaly, physical development lag, specific dislocations of the facial skeleton, as well as frequent recurrent infections, increased sensitivity to radiation, and predisposition to the implementation of malignant lymphoid processes. Most patients of Slavic origin have a homozygous mutation in the NBS1 gene. About 80-90% of patients with primary immunodeficiencies remain undiagnosed, including children with Nijmegen Syndrome.

Objective: It was carried out an assessment of 6 patients with diagnosed Nijmegen syndrome physical development in the Ternopil region (5 boys and 1 girl, aged from 1 to 12).

Methods: Assessment of physical development was conducted in accordance with:

- norms proposed by WHO (Order No. 149, Ukraine’s Ministry of Health, 2008) for children under 5 years old;

- censored schedules of growth and mass (Order No. 254, Ukraine’s Ministry of Health of Ukraine, 2006) for children over 5 years old.

Results: All patients had a delay in body weight and growth since birth. There was also a significant difference in head circumference – microcephaly, detected in all patients. It was the main diagnostic criterion for the Nijmegen Syndrome (besides genetic confirmation).

Conclusion: The delay in weight and growth is one of ten alarming primary immunodeficiencies proposed by a number of primary immunodeficiency foundations in the world. This indicator, together with microcephaly, is a clinical criterion for the Nijmegen Syndrome and can help to diagnose the problem in the early period. Child Nijmegen Syndrome is subject to compulsory early diagnosis among the Ukrainian population due to its prevalence in Slovyans. Proper diagnosis of this pathology will allow to start therapy faster, prevent complications and reduce health care costs.

701

Allergy & Immunology

Applying LEAP Recommendations In Clinical Practice: Not As Straightforward As It Seems

Gillian Hendriks ¹ , Claudine Bryson ¹ , David Lacy ¹

¹ Department of Paediatrics, Wirral University Hospital NHS Foundation Trust, UK

Background: Following the revolutionary results from the LEAP (Learning Early about Peanut Allergy) study advising the early introduction of peanuts to children at high risk of this allergy, clinicians and allergists have been trying to implement this safely into clinical practice, with the ultimate goal of reversing the trend of rising peanut allergy within the population. However, despite good intentions and well-thought through methods, this does not always go to plan.

Method: We discuss a 13-month old girl with severe eczema who has a negative skin prick test, IgE and component blood tests to peanut, and is suitably advised to perform an oral peanut challenge at home. Unfortunately, Mum subsequently reports symptoms of widespread urticaria and watery eyes on ingestion of a small amount of peanut butter. Despite the likelihood that this is an allergic reaction to peanut, because of the discrepancy between her tests and her home challenge, parents request to proceed with an hospital-based oral food challenge.

Conclusion: Applying evidenced-based knowledge within clinical practice may not be as straightforward as clinicians would envisage. In order to work together and made a significant reduction in prevalence of peanut allergy particularly in the UK, clearer national guidelines or recommendations need to be developed to guide clinicians towards the safe introduction of peanuts in children with high risk of developing this allergy.

994

Allergy & Immunology

Kawasaki Disease Shock Syndrome; a Different Subtype of Kawasaki Disease?

Andrea Iglesias Amaya ¹ , Giselle Quezada Ortega ² , Marycarmen Godínez Victoria ² , Chiharu Murata ¹ , Mónica Adriana Montoya Guzman ² , María Elisa Drago Serrano ³ , Rafael Campos Rodríguez ² , Luis Adrián Rosales Hernández ¹ , Marco Antonio Yamazaki Nakashimada ¹

¹ Department of Clinical Immunology, Instituto Nacional de Pediatría, Mexico ² Instituto Politécnico Nacional, Mexico ³ Biological production area, Universidad Autónoma Metropolitana, Mexico

Background. Super antigen (SAg) is suspected to be responsible of Kawasaki Disease Shock Syndrome (KDSS) which is an aggressive complication of Kawasaki Disease (KD). We hypothesize that KDSS is associated with the activation of TCRVβ2 and TCRVβ8 lymphocytes through the presence of SAg from bacteria that colonize the gastrointestinal tract.

Objective. To compare the expression of TCRVβ2 and Vβ8 receptor variants in lymphocytes of the KDSS and KD groups.

Methods. A case-control study was performed in patients admitted in the National Institute of Pediatrics between January 2018 and July 2019. Case group (n=8), patients with KDSS; control group (n=56), patients with KD without shock. The percentage (%) of CD4 and CD8 T cells expressing TCRVβ2 and TCRVβ8 as well the activated lymphocytes CD25+ were determined in blood samples by flow cytofluorometry. Wilcoxon Rank-Sum Test was used to compare both groups.

Results. The expression of variants CD3+CD4+TCRVβ2+ and CD3+CD8+TCRVβ8+CD25+ was significantly higher in the KDSS group, in comparison to non-shock KD group with a median [Q1, Q3]: 27.7 [4.0, 53.2] vs. 2.2 [0.3, 9.4] (P= 0.016) and 13.7 [3.9, 56.3] vs. 2.6 [0.5, 11.2] (P=0.028), respectively.

Conclusion. These preliminary data suggests that a SAg is triggering the excessive response observed in patients with KDSS. Continuing this study with a bigger sample size will be necessary.

620

Allergy & Immunology

Increased Skin Reactivity to Histamine along with an Increase of Body Mass Index in Korean Children: 2 Years Follow Up Study

Suk Won Chang ¹ , Jin-A Park ¹ , Ju Wan Kang ¹ , Gil-Chai Lim ¹

¹ Otorhinolaryngology, Jeju National University School of Medicine, South Korea

Background: A skin prick test is the most widely used test to diagnose allergic diseases. However, it is not known much about the effect of the body mass index on skin reactivity. Therefore, we aim to investigate the changes in skin reactivity to histamine and allergens according to the changes in body mass index (BMI) in Korean children.

Methods: The data of 230 children were used in this analysis. And, the data were collected in 2010 and 2012, respectively. Skin prick test was performed for 26 aeroallergens and other variables were obtained, including sex, age, BMI, parental allergy history, and parental smoking. Multivariate analysis was used to confirm the association between the changes in skin reactivity and body mass index.

Results: Mean size of skin wheal induced histamine and BMI were increased for two years. Changes in skin wheal induced by histamine showed significant correlation with changes in BMI (Spearman’s Rho=0.209, P-value 0.001). However, changes in the size of the skin response to Dermatophagoides farinae (Df) and Dermatophagoides pteronyssinus (Dp) did not correlate with changes in BMI.

Conclusion: The magnitude of skin response to histamine was correlated with an increase in body mass index. However, the wheal size for Df and Dp was not related to the change in the BMI. The effects of BMI on the interpretation of skin prick test should be considered.

230

Allergy & Immunology

Drug Induced Hypersensitivity Syndrome (DIHS) in a 12 Month Old Child

Ricardo Loureiro ² , Luisa Prada ¹ , Isis Monteiro ¹ , Ana Neves ¹

¹ Pediatric Allergology Unit, Santa Maria University Hospital - Lisbon University Hospital Center, Portugal ² Pediatric Unit, Federal University of Rio de Janeiro, Brazil

Introduction: Drug rash with eosinophilia and systemic symptoms syndrome (DRESS)/DIHS is a complex syndrome characterized by a broad spectrum of clinical features, like fever, lymphadenopathy, cutaneous manifestations and visceral involvement, eosinophilia and atypical lymphocytosis.

Beta-lactams, anticonvulsants, allopurinol, sulfonamides are culprit drugs than can trigger this delayed hypersensitivity mechanism. Herpes virus-drug interactions are sometimes observed.

A 12-month-old female baby, with congenital heart disease and chromosomal abnormality (deletion of short arm of chromosome 8) was submitted to undergo cardiac surgery.

On day 6 post-surgery she was prescribed with intravenous vancomycin and gentamycin, for infection.

Due to loss of intravenous line, on day 7 post-surgery, a switch to oral amoxicillin and clavulanic acid was done. On day 8 of antibiotic, she presented an urticarial maculopapular rash with facial edema and fever, hepatomegaly and lymphadenopathy, associated with laboratorial abnormalities 10% atypical lymphocytes, and elevated AST 429 U/L and ALT 621U/L, suggestive of a mononucleosic syndrome. On day 11 of amoxicillin and clavulanic acid, this antibiotic was suspended. Epstein Barr virus, cytomegalovirus and toxoplasmosis were IgG positive. Parainfluenza virus type 3 was also positive. HHV 6 and 7 serologies and measles virus RNA were negative. On day 18 of admission, the child was seriously ill and vancomycin and ceftriaxone were again started. On day 29 post-surgery, endocarditis was confirmed by cardiac ultrasound, and daptomycin, cefepime and ciprofloxacin was started with clinical improvement. The baby was discharged on day 48 post-surgery, a subtle rash in her legs was still observed.

Four months later, she was inadvertly prescribed amoxicillin and clavulanic acid for an upper respiratory infection. On the 3^rd day of antibiotic, she presented again with an urticarial rash that was promptly suspended.

Conclusions: The diagnosis of DRESS/DIHS was proposed, based on RegiSCAR (European registry of severe cutaneous adverse reactions to drugs) criteria.

765

Allergy & Immunology

The Benefit Supplementing with Vitamin D and Omega3 in Atopic Dermatitis in Children

Alina Murgu ¹ , Anca Chiriac ² , Irina Criscov ¹ , Paula Popovici ¹ , Alice Azoicai ¹ , Alice Popescu ³

¹ Pediatrics, Immuno-Allergology, Children Emergency Hospital "St.Mary", Romania ² Dermatology, Recovery and Balneology Hospital, Romania ³ Pediatric, Privat Pediatric Office, Romania

Atopic dermatitis through clinical expression and evolution is a chronic inflammatory allergic disabling disorder with a major impact on the quality of life of the child and its family. The nutritional assistance of these patients becomes essential to allow for normal growth and development. Supplementation with vitamin D and Omega 3 seems to be beneficial by the complex immunomodulatory and the anti-inflammatory action, by modifying the cytokine production but also by the antiinfectious role

Objective: To assess the benefit of dietary supplementation with vitamin D and Omega 3 on the evolution of atopic dermatitis in the child.

Method: 47 children (0-2 years) diagnosed with atopic dermatitis with varying severity were studied. Two groups were selected: group A -23 cases; Group B - 24 cases. Serum vitamin D levels in both groups were normal. Supplementation with vitamin D3 (1500UI / day) and Omega3 was done only in group A. Group B received the usual dose of Vitamin D3 for the rickets prophylaxis 500 IU / day. The monitoring was performed for 6 months. The treatment for atopic dermatitis during supplementation with the vitamin D and Omega 3 was only with non-cortisone cutaneous topicals and no one calcineurin inhibitors topic.

Results: Initial: group A -SCORAD≤ 15 -8/23; SCORAD: 15-40-12 / 23; SCORAD≥40 -3/23; group B -SCORAD≤ 15 -9/24; SCORAD: 15-40-13 / 24; SCORAD≥40 -2/24; incidence of skin infections: group A-37%; group B-35%; after 6 months: group A-SCORAD≤ 15 -15/23; SCORAD: 15-40-7 / 23; SCORAD≥40 -1/23; group B -SCORAD≤ 15 -10/24; SCORAD: 15-40-12 / 24; SCORAD≥40 -2/24; incidence of cutaneous infections: group A-21%; group B-36%;

Conclusions: Supplementing the diet of patients with atopic dermatitis with Vitamin D and Omega 3 improves pruritus, dryness and skin rash, while reducing the rate of cutaneous infections.

386

Allergy & Immunology

ACE Inhibitors: An unusual Cause of Pediatric Angioedema

Goncalo Vale ¹ , Maria São Pedro ¹ , Rita Matos Parreira ¹ , Mariana Simões ¹ , Patrícia Pais ¹ , Joana Extreia ¹

¹ Pediatrics, Centro Hospitalar Barreiro-Montijo, Portugal

Background: Angiotensin converting enzyme inhibitors (ACEI) are widely used to treat hypertension in adults. The use of these drugs in pediatric age is increasing due to the higher prevalence of obesity and its co-morbidities’.

Objective: To alert pediatric community about angioedema as ACEI side effect.

Methods: Report of clinical case from a suburban hospital in Portugal.

Clinical Report: Fifteen year-old african descendent female with morbid obesity, hypertension, diabetes mellitus type 2 followed in cardiovascular risk outpatient clinic. Treated with lisinopril since December 2017.

During hospital admission in January 2019, she developed labial and tongue edema followed by asymmetric periorbital involvement, without itching nor urticaria, unresponsive to high dose hydroxyzine. Lisinopril was discontinued and no other therapeutic measures were taken. Clinical manifestations gradually disappeared during the following five days. The patient reported a similar episode five months before that resolved spontaneously, without therapy changes.

Conclusion: In adults, angioedema is an unusual but well-recognized side effect of ACEI therapy, up to five times greater in African descents. However, in the “pediatric world”, physicians are less familiar and therefore less alert to possible ACEI side effects. Although benign and self-limited, ACEI induced angioedema can be potentially fatal, therefore physicians should consider this possibility when evaluating patients with acute or recurrent angioedema.

156

Allergy & Immunology

Self and Proxy Reported Quality of Life of Children with Asthma

Vaida Taminskiene ¹ , Egle Vaitkaitiene ² , Arunas Valiulis ^1,3

¹ Department of Public Health, Institute of Health Science, Faculty of Medicine, Vilnius University, Lithuania ² Medical Academy, Lithuanian University of Health Sciences, Lithuania ³ Clinic of Children’s Diseases, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, Lithuania

Background: Asthma is a global public health program. It affects patients of different age and is one of the most prevalent chronic diseases among children.

Objective: The aim of our study was to evaluate the quality of life (QoL) of children with asthma by both self- and proxy-reports.

Methods: A total of 527 Lithuanian children with asthma and their parents were enrolled into a cross-sectional study. Participants of the study were asked to fill in anonymous questionnaires during scheduled outpatient visits to pediatric pulmonologist. QoL of children with asthma was assessed using PedsQL Asthma Module consisting of 2 scales and 22 questions. Age-appropriate versions of the questionnaire were completed by children and their parents. Scores for each scale and overall score were calculated and ranged from 0 to 100 where higher score referred to better QoL.

Results: The age of children enrolled into this study varied from 2 to 17 years and the mean age was 8.4 (±4.3) years. A total of 63.2% of respondents were boys. Children evaluated their asthma related QoL better compared to parents’ evaluation (median score 77.3 (68.2; 86.4) and 75.0 (63.6; 86.4) respectively, p=0.045). The differences between parent and children assessment were the highest at the oldest age group. The most important factors, associated with the QoL of children with asthma by both self- and proxy-reports were asthma severity and control as well as shortness of breath. Parent reported QoL was also related with child’s general health and presence of concomitant allergies.

Conclusions: Children with asthma assess their QoL better compared to their parents. The differences in the assessment were the highest at the oldest age group of children and should be taken into consideration during the preparation of individual asthma control plan.

1013

Critical / Emergency Care

Traumatic Thoracic Aortic Transection After a High-Energy Trauma

Mariana Adriao ¹ , Elson Salgueiro ² , Maria José Oliveira ³ , Amélia Moreira ³ , Augusto Ribeiro ³

¹ Serviço de Pediatria, Centro Hospitalar Universitário de São João, Portugal ² Serviço de Cirurgia Cardiotorácica, Centro Hospitalar Universitário de São João, Portugal ³ Serviço de Medicina Intensiva Pediátrica, Centro Hospitalar Universitário de São João, Portugal

Background: Deceleration trauma resulting in thoracic aortic contained rupture is uncommon and associated with a high prehospital mortality. Less than 25% of patients with blunt thoracic aortic injury survive long enough to reach a hospital. Radiological plays an important role in diagnosis and accurate classification of the type of dissection, in order to plan the intervention and optimize the outcome.

Objective: The authors aim to report a rare case of blunt thoracic aortic injury successfully corrected and share the correspondent unique imaging.

Case Report: A 17-year-old high energy accident victim with several traumatic lesions arrived in the emergency room. He was hemodynamically stable and had a Glasgow Coma Scale of 15/15. He presented diminished breath sounds on the left hemithorax and gross hematuria. Chest radiograph revealed left hemidiaphragmatic rupture with stomach herniation and mediastinal enlargement. A thoracic CT scan confirmed visceral herniation through the ruptured diaphragm, fracture of four left ribs and a contained aortic rupture located at the aortic isthmus, associated with perivascular hematoma – type IV. Endovascular treatment was considered unsuitable due to unfavorable anatomical characteristics. Therefore, the patient was submitted to open descending aorta replacement by a vascular prothesis. Distal aortic perfusion was maintained by a Gott shunt. Simultaneously, diaphragm laceration was directly sutured. Post-operative radiological revaluation revealed good resolution of both lesions.

Due to acute renal injury caused by severe rhabdomyolysis, continuous venovenous hemodiafiltration was required for 25 days. Regarding outcome, the adolescent was discharged home 44 days after hospital admission, without any severe sequelae.

Conclusion: This clinical case highlights the importance of considering different types of internal hemorrhage in cases of trauma, particularly life-threatening lesions resulting from blunt trauma. In this particular case, the patient was hemodynamically stable at all times, which was only possible due to the containment of the hemorrhage to the mediastinum.

495

Critical / Emergency Care

Poisoning and Mucosal Burning by Laundry Detergent Capsules: A Case Report

Jose Andrade Guerrero¹, Virginia Santana Rojo¹, Maria Jose Sanchez Toro¹, Celia Fabra Garrido¹, Isabel Gonzalez Bertolin¹, Begoña de Miguel Lavisier¹, Maria Fernanda Pedrero Escalas², Francisco Javier Aracil Santos³

¹ Pediatric Emergency, Hospital Universitario La Paz, Spain

² Pediatric Otorhinolaryngology, Hospital Universitario La Paz, Spain

³ General Pediatrics, Hospital Universitario La Paz, Spain

Background: One of the main sources of poisoning in young children are cleaning products. Among the newest ones, we find laundry detergent capsules (LDC). Because their higher concentration of detergent serious clinical manifestation can be observed.

We present a case of a 12-month-old boy who bit an LDC (Ariel 3in1), and developed metabolic acidosis, digestive and respiratory impairment.

Case Presentation: A healthy 12-month-old boy, was brought to Emergency Room vomiting, coughing and dysphonia after biting an LDC. We contacted the National Institute of Toxicology of Spain who told us the main risk of this ingestion is metabolic acidosis, but not chemical mucosal burning. In the physical examination he was irritable with presence of respiratory distress and inspiratory stridor. Laboratory tests on admission revealed: Hemoglobin: 11.4g/dL, WBC 29400/μL (neutrophils 57%). The blood gases showed a metabolic acidosis (pH 7.29, HCO3: 15mmol/L, pCO2: 40mmHg, BE: -13mmol/L). Renal function was normal. A chest X-ray revealed bilateral peribronchial thickening, predominantly at the left base. He was initially treated with dexamethasone, nebulized adrenaline, salbutamol, oxygen and bicarbonate perfusion, which was maintained for 3 days. On the second day, an upper endoscopy showed caustic ulcerations in epiglottis and subglottic regions. Esophageal endoscopy did not show any damage. Hemoglobin gradually dropped to 8g/L at 3^rd day. Digestive hemorrhage was suspected and treated with omeprazole.

At discharge respiratory symptoms and metabolic acidosis were solved with no digestive symptoms. Three months after an upper gastrointestinal series showed a membrane in the middle third of the esophagus.

Conclusions: The main component of the Ariel 3in1 is Dodecylbenzenesulfonte which may produce gastrointestinal burning. However, combining it with other component, Propylene-gycol, the toxicity may enhance, and appear metabolic acidosis. When bitten by children can burst and spray the content in the respiratory and GI systems, causing mucosal damage leading to several complications.

733

Critical / Emergency Care

Pediatric Urgent Care – A Three-year Institutional Experience

Magdy Attia ¹

¹ Pediatrics, Division of Urgent Care, Nemours Alfred I duPont Hospital for Children, USA

Background: Pediatric Emergency Departments (PED) evaluate a large number of sub-acute conditions. PED is convenient but expensive resource for sub-acute care to providers and patients alike. Pediatric Urgent Care (PUC) is rapidly reshaping the landscape in delivering subacute care for children. This report is to share our experience.

Methods: Data analyzed included demographics, chief complaints (CCs), throughput, cost, satisfaction and quality. The analysis describes basic operational statistics and compared CCs and volumes during different “seasons”. We compared PUC and PED length of visit (LOV) and cost. Patient satisfaction was measured by 5-point Likert scale. Data are presented as percentages, means or p values.

Results: We analyzed 15,482 visits. Daily average volume; 18.4 patient (range 7-67). Visits increased 60% from year 1 to year 2 and a 25 % in year 3. Weekends accounted for 39% of the visits. Fall/winter season was busier and Sundays were most crowded. The first hour on Sunday mornings had the highest arrival rate. The least arrivals were in the last hour of weeknights. 51% were males. Age average was 12.9 years (SD: + 9.9). Most common CCs were respiratory infection, fever, extremity injury and sore throat; 14%, 13%, 12%, and 10% respectively. There was no significant difference between CCs during fall/winter with more infectious etiologies compared to spring/summer with more injures (P0.5). PUC LOV was 52 minutes and 117 in PED. Percent of 48-hours returns to PUC was 1.1 % (95% CI: 0.8-1.4) and percent of patients transferred to PED was 1.5% (95% CI:-1.1-1.9). Adherence to clinical standard work was 97%. Satisfaction score was 83.5% using score of 5. PUC charges for similar visit level was 47-61 % lower than PED.

Conclusions: PUC are efficient and economical alternative to PED. We described one institution’s experience that could prove valuable benchmark to similar practices.

548

Critical / Emergency Care

Unintentional Intoxications in Children under 4 Years Old: A Current Reality?

Denise Banganho ¹ , Diana Silva ¹ , Inês Ferreira ¹ , Ana Gomes da Silva ¹ , Teresa Gouveia ¹

¹ Department of Pediatrics, Hospital de São Bernardo – Centro Hospitalar de Setúbal, Portugal

Background: Young children are particularly susceptible to intoxications due to the natural need of environmental exploration and inability to measure risks. Despite several preventing efforts, intoxications remain a significant cause of admission to the pediatric emergency department (PED).

Objectives: Characterize childhood intoxication cases in the PED and develop prevention strategies.

Methods: Retrospective analysis of clinical cases of children under 4 years old attending to the PED during 10 years.

Results: Between 2008-2017, 383 children with unintentional intoxications attended the PED and accounted for 0,1% of all emergency visits during the study period. The median age was 2.1 years (±1.0 years); 54.3% (n=208) were female. Ingestion of drugs were involved in 52.5% (n=201) and antihistamines were the most frequent drug class. Domestic products were the second intoxication agent (36%, n=138) followed by carbon monoxide (5.7%, n=22), poisons (4.4%, n=17) and illicit drugs (0.8%, n=3). Forty percent (40.7%, n=156) of the intoxications occurred in the afternoon, 68.1% (n=261) during weekdays. 99.7% (n=382) intoxications occurred at home. A total of 50.4% (n=193) of patients were admitted less than 1 hour after exposure to the toxic agent and only 15.1% were transported to the hospital through the National Emergency Service. Most children were asymptomatic (n=224). In symptomatic patients, gastrointestinal manifestations were the most frequent (58.4%, n=93). The National Poisoning and Intoxication Center was contacted in 82.2% (n=315) of the cases. Gastrointestinal decontamination was performed in 30.3%. Four children underwent hospitalization for more than 36 hours. There were no admissions to the intensive care unit or mortality.

Conclusions: The number of children with unintentional intoxication did not change significantly over the last 10 years. Ingestion of drugs that belong to family members accounts for approximately half of the cases. It remains important to ensure prevention strategies in the community.

530

Critical / Emergency Care

Acute Appendicitis in Children: Postoperative Evolution According to Risk Factors; Single-Centre Retrospective Review

Maria Bauluz-Barcena ¹ , Antia Fiel-Ozores ¹ , Jessica Lopez-Lopez ² , Pilar Fernández-Eire ²

¹ Pediatrics, Hospital Alvaro Cunqueiro, Spain

² Pediatric Surgery, Hospital Alvaro Cunqueiro, Spain

Background: Abdominal pain is a frequent complaint in Pediatric Primary Care as well as in the Emergency Department. Acute appendicitis (AA) is the main etiology of surgical abdominal pain in children.

Objectives: Our aim is to describe and analyze the clinical characteristics of our patients who have undergone surgery on suspicion of AA. Secondly, to describe AA evolution according to some prognosis factors.

Methods: Retrospective, descriptive and analytical study through review of electronic clinical records. Patients from 0 to 14 years old who have undergone surgery for suspicion of AA, are included. Study period: 8 months. Setting: tertiary hospital.

Results: Sample size = 96. Median age = 10`8 years. Male sex: 64.6%. The most frequent symptoms in our study are: pain in low right quadrant (79.2%), nausea/vomiting (72.9%), fever (32.3%) and hyporexia (22.9%). Preferred diagnostic method is ultrasound (performed in 91.6%). Most common intraoperative findings are: phlegmonous AA (37.5%), gangrenous AA (40.6%), plastron (10.4%). 37.5% appendices are retrocecal. We have analyzed the incidence of complications (days of total admission, admission to pediatric intensive care, need for a central catheter or parenteral nutrition, need of surgical reintervention) in children under 4 years of age, in gangrenous AA, in those with ultrasonography data suggesting complicated AA, and those with increased CRP.

Conclusions: Acute appendicitis is the main surgical diagnosis of abdominal pain in pediatric age. According to literature, some factors are known to be related to worse prognosis, such as: age under 4 years old, gangrenous or perforate AA and CRP increase. However, we have not found a statistically significant association in this respect.

394

Critical / Emergency Care

The Role of Extracorporeal Membrane Oxygenation Support in Malignant Infantile Pertussis

Francisca Calheiros-Trigo ¹ , Lurdes Lisboa ² , Marta João Silva ² , Roberto Roncon-Albuquerque Jr ³ , Augusto Ribeiro ²

¹ Department of Pediatrics, Hospital de Braga, Portugal

² Pediatric Intensive Care Unit, Centro Hospitalar Universitário de S. João, Portugal

³ Department of Intensive Care Medicine, Centro Hospitalar Universitário de S. João, Portugal

Background: Pertussis, or whooping cough, is an acute respiratory infection caused by Bordetella pertussis and Bordetella parapertussis. Malignant pertussis (acute respiratory distress syndrome (ARDS), hyperleukocytosis and pulmonary hypertension) is a severe form of pertussis, more common in young, non-vaccinated children, and almost always a fatal disease. Extracorporeal membrane oxygenation (ECMO) is used as an emergency treatment for children with life-threatening pertussis. However, the success rate is low, with only 30% survival rate.

Method: We report a clinical case of a newborn admitted at pediatric intensive care unit (PICU) due to malignant pertussis.

Results: A female newborn (28-days of age) with pertussis as a result of B. pertussis infection was admitted in PICU due to hyperleukocytosis (92030/uL) and worsening respiratory distress. Exchange transfusion was performed with mild improvement. Cardiac ultrasound revealed pulmonary hypertension. By day-2 there was clinical worsening with severe ARDS. After failure of conventional ventilation and high-frequency oscillatory ventilation, cannulation of internal jugular vein and common carotid artery was performed and venoarterial ECMO support was instituted. Due to anasarca, dialytic therapy was maintained between days 9 and 32. Serial transfontanelar ultrasounds were performed to access for brain complications. By day-20 ultrasound revealed brain hemorrhage and epileptic activity was noted, with abnormal eletroencephalogram. Antiepileptic therapy was started, with good response. After 41 days of ECMO support the patient was weaned off ECMO, and extubated to non-invasive ventilation 14 days later. She was discharged from PICU at day-63 in spontaneous ventilation. She was discharged home at day-105.

Conclusion: In this patient with malignant pertussis, ECMO was performed for cardiopulmonary support and rescued the infant. During ECMO support, mechanical ventilation with protective settings (high positive end-expiratory pressure and low tidal volumes) provided the opportunity for lung recovery. Despite complications (minor brain hemorrhage) the patient recovery with minor sequelae poses a good prognosis.

760

Critical / Emergency Care

Pediatric Hand Injuries Presenting to the Emergency Department: A 6-year Cross-sectional Study in Korea

Joong Wan Park¹, Do Kyun Kim¹, Ik Chang Choi¹, Jae Yun Jung¹, Se Uk Lee², Soyun Hwang¹

¹ Emergency Medicine, Seoul National University Hospital, South Korea

² Emergency Medicine, Seoul Metropolitan Government Seoul National University Boramae Medical Center, South Korea

Objective: The objective of this study was to evaluate the clinical characteristics of pediatric hand injuries presenting to the emergency department (ED) in Korea and to assess the risk factors associated with severe hand injuries requiring hospitalization or surgery.

Methods: A multicentre cross-sectional study was conducted using the emergency department (ED)-based Injury In-depth Surveillance (EDIIS) registry in Korea between 2011 and 2016. A multivariable logistic regression model was used to obtain the adjusted odds ratios (aORs) for the factors associated with severe pediatric hand injuries.

Results: Among 1,537,617 injured patients, 40,672 (26.5 per 1,000 injured patients) presented to the ED for hand injuries. The prevalence of pediatric hand injuries was highest in teenager (9.9%) and increased with age. Overall and in each age group, boys were in the majority. In infant, toddler, and pre-schooler, crushing is the most common injury mechanism, while penetrating injury were most common in schooler and teenager. Door related injuries were the most common cause in infant, toddler, and preschooler. Knife related injuries were the most common cause in schooler and teenager. A total of 2,344 (5.8%) were severe injuries requiring hospitalization or surgery.

Aging (aOR: 1.06, 95% CI: 1.06−1.07), male (aOR: 1.33, 95% CI: 1.22−1.46), finger injury (aOR: 1.26, 95% CI: 1.14−1.39), crushing injury (aOR: 1.35, 95% CI: 1.15−1.57), and injury by machine (aOR: 3.91, 95% CI: 2.66−5.75) were associated with severe hand injury.

Conclusion: This study provides a comprehensive overview of the epidemiology of hand injuries in the pediatric population. Preventive strategies are needed to prevent severe hand injuries in children.

898

Critical / Emergency Care

Clinical Validation of Affordable, Reusable Novel EKG Monitor for Children in Low-Resource Settings

Judy Chen ¹ , Andre Muelenaer ^2,3 , John Bird ⁴

¹ Virginia Tech Carilion School of Medicine, USA

² Department of Pediatrics, Carilion Clinic, USA

³ Department of Biomedical Engineering and Mechanics, Virginia Polytechnic Institute, USA

⁴ Cardinal Mechtronics, USA

Background: Low-income countries severely lack adequate access to devices that accurately measure vital signs which may affect mortality outcome for pediatric patients. Developing an efficient and affordable devices that can accurately determine vital signs can contribute greatly to diagnosing critical pediatric conditions, leading to faster time to appropriate treatment. A team of engineers, industrial designers, and medical professionals developed an affordable, reusable EKG monitor prototype using carbon electrodes.

Objective: The objective of study is to validate the prototype’s accuracy compared to a standard EKG monitor.

Methods: 21 healthy subjects, ages newborn to 60 months, were recruited from Carilion Children’s General Pediatrics Clinic. Prototype device and standard EKG device were simultaneously attached to subject for 1-2 minutes. Bland-Altman method was used to evaluate if agreement existed between both devices with heartbeats counted obtained per 10-second interval (hpi) as N=1.

Results: Prototype was able to detect subject’s heartbeat and display distinct EKG waveforms. Loss of EKG detection from prototype was observed during loss of skin contact with carbon electrodes from movement.

Mean of differences between the two methods clinically translates to signficant difference of 45 heartbeats per minute. Range between limits of agreement demonstrates clinically significant discrepancy in prototype’s ability to consistently detect heart rate, presumably from loss of skin contact. However, 35% of data show difference between -2.2 to 0.4hpi between the two methods, indicating the prototype can detect heart rate similarly to the standard EKG in presumed instances of constant skin contact.

Conclusion: For our prototype, we were able to develop a low-cost, reusable device that can detect heart rate in children and reproduce similar heart rates as the standard EKG monitor, but inconsistently, presumably secondary to loss of skin contact with the carbon electrodes. Improvements to optimize carbon electrode contact with skin will be considered for future design.

952

Critical / Emergency Care

Audit of Routine Blood Tests on Children’s Intensive Care Unit, Leicester Royal Infirmary

Daniel Crankshaw ¹ , Jennirfer Mann ¹ , Simon Robinson ¹

¹ Children’s Intensive Care Unit, Leicester Royal Infirmary, UK

Aim: Over an 8 week period, an audit was performed to analyse the blood tests performed overnight on paediatric inpatients on our unit with the aim of identifying whether more samples are taken than required and the financial impact of this.

Methods: This audit assessed essential (those blood tests which the consultant of the week recommended be taken overnight) versus those actually requested by the paediatric registrar working the night shift. The consultant of the day was asked to complete a short proforma, recording which essential bloods were needed overnight. This consultant recommendation was blinded from the registrar. We then retrospectively looked through iLab to record which bloods had actually been sent overnight.

Findings: Data was collected for 62 patients over 16 weekdays on CICU. In total, 165 tests were performed, with 90 of these classed as essential and 75 (45%) as non - essential tests. This resulted in avoidable expenditure of £123.74 (45 %.) Coagulation, urea and electrolytes and liver function tests were the areas of most non-essential expenditure. This is similar to results found when this audit was performed on the Paediatric Intensive Care Unit at Glenfield Hospital, where 115/1059 (48%) were found to be non-essential, resulting in avoidable expenditure of £647.64 (44 %.)

Conclusions: This audit has now been performed at both CICU’s within our trust and it demonstrates a lack of correlation between which tests are thought to be essential by the paediatric intensive care consultants and which tests are actually being performed by the paediatric registrars covering the units overnight. Therefore, children are likely to be subjected to additional blood investigations which are non-essential to their care and also has a financial impact upon the department. Further consideration of an appropriate quality improvement initiative is needed to address these discrepancies.

422

Critical / Emergency Care

Validity and Reliability of the Brazilian Portuguese Version of the Pediatric Confusion Assessment Method for the Intensive Care Unit (pCAM-ICU)

Roberta Castro ¹ , Maria Clara Magalhães-Barbosa ³ , Arnaldo Prata-Barbosa ³ , Antonio Cunha ^2,3 , Elie Cheniaux ^1,2 , Heidi Smith ⁴

¹ Pediatrics, Universidade do Estado do Rio de Janeiro (UERJ), Brazil ² Pediatrics, Universidade Federal do Rio de Janeiro (UFRJ), Brazil ³ Pediatrics, Instituto D’Or de Pesquisa e Ensino (IDOR), Brazil ⁴ Pediatrics, Vanderbilt University Medical Center, USA

Objectives: To determine the validity and reliability of the Brazilian Portuguese Pediatric Confusion Assessment Method for the Intensive Care Unit (BRpCAM-ICU) for diagnosing delirium in patients with chronological and developmental ages from 5 to 17 years in Brazilian Pediatric Intensive Care Units (PICUs).

Methods: Prospective, cross-sectional study performed in eight PICUs, with 116 patients, 5–17 years of age, without developmental delay, submitted to mechanical ventilation or not. To assess the interobserver reliability, two previously trained researchers concomitantly applied the BRpCAM-ICU and independently rated the same patient. To assess the criterion validity, a pediatric neurologist or psychiatrist, blinded to the BRpCAM-ICU assessments, evaluated the same patient within 30 minutes, using the Diagnostic and Statistical Manual of Mental Disorders Fifth Edition – DSM-5, considered the reference standard.

Results: 149 paired assessments were included (some patients had more than one). Delirium was diagnosed in 11 of 149 (7%) assessments, or 8 of 116 (7%) patients, using both the BRpCAM-ICU and the DSM-5. There was one false positive and one false negative diagnosis, which resulted in 90.9% sensitivity (95%CI, 58.7–99.8%) and 99.3% specificity (95%CI, 96–100%) for the BRpCAM-ICU. The interrater reliability was considered almost perfect (κ = 1.0).

Conclusions: The BRpCAM-ICU is a valid and reliable tool for diagnosing delirium in pediatric patients 5–17 years of age in Brazilian PICUs. The implementation of this tool may be useful to reduce underdiagnosis, ensure monitoring and earlier intervention, provide a better prognosis, and improve research on delirium in this age group in Brazil.

521

Critical / Emergency Care

Chest Pain in Children and Adolescents: Reality of an Emergency Department

Ana Barbara De Matos ¹ , Nelia Gaspar ¹ , Sara Ferreira ¹ , Julieta Morais ¹

¹ Paediatric Service, Centro Hospitalar Médio Tejo, Portugal

Background: Chest pain is a common complaint in children/adolescents visiting the emergency department (ED). The existence of underlying heart disease is rarely verified. It is mostly idiopathic, and in adolescence the psychogenic etiology assumes greater expressiveness.

Objective and Methods: Retrospective analysis of the clinical processes of patients who visited the ED between December 2017 and May 2018 with chest pain. Cases of traumatic etiology were excluded.

Results: A total of 175 children/adolescents (52% girls; median age 13 years) attended the ED. Five percent had cardiovascular antecedents, and 7% psychiatric. Regarding pain characteristics, the majority lasted between 1 hour and 1 day (27%), with preferential retrosternal location (18%) or in the left hemithorax (18%). Associated symptoms occurred in 69% of patients, including 5% of palpitations and 5% of paresthesias in the left upper limb. Physical examination revealed no abnormalities in 39% of patients, 25% reported chest wall tenderness and 5% had altered cardiac auscultation. Eleven percent reported at least one stressful event associated with the onset of chest pain. Fifty-seven percent had chest X-ray (16% altered), 23% electrocardiogram (7% altered), 51% cardiac enzymes (2% altered) and 32% did not perform any additional investigation. Regarding the final diagnosis, 38% was idiopathic, 29% respiratory, 17% musculoskeletal, 14% psychogenic and 2% cardiac. Concerning psychogenic pain, there was a statistically significant relationship with the adolescence phase, female sex and the presence of previous stressful events (p 0.05). Two percent of the patients were admitted in the pediatric unit and 2% were transferred to a cardiovascular reference center.

Conclusion: The number of children/adolescents who were admitted due to chest pain, highlights the importance of the characterization of this population. Cases of cardiac etiology are rare and it is therefore essential to give young people and families a sense of security in the absence of serious illness.

1002

Critical / Emergency Care

Utility of Chest Radiographs (CXR) in Paediatric Critical Care Setting

Ramona Dumitru ¹ , Paula Avram ¹ , Konstantinos Dimitriades ¹

¹ Paediatric Critical Care Unit, Royal London Hospital, UK

Background and aims: XRays have become frequently used imaging tools in the Critical Care setting. CXRs (Chest Xrays) are used either to support clinical decision making and result in therapeutic changes or are used to confirm adequacy of current interventions and remain unchanged. This is a retrospective study aimed at evaluating the number of CXRs our patients receive and how the management of patients is influenced by such CXR.

Methods: All patients admitted during Oct–Nov 2017 on Paediatric Critical Care Unit at Royal London Hospital who have undergone at least one CXR per admission in the PCCU. Patients who have received only other XRays – such as abdominal, neck, pelvis, etc. will not be included in the study.

Clinical Data collection: Age of patient, PIM Score, Primary Diagnosis, Length of stay, Type of ventilation – invasive / non-invasive, Number of CXR received during admission, Indication for request, Routine – (e.g. Line/ET check), Urgent Xrays – (e.g. Clinical suspicion of pneumothorax/effusion), Change in management following CXR

Results: There have been 91 patients admitted between 01.10 -30.11.17. Of these, 74 had at least one CXR done. Total 141 of xrays. Average length of admission - 5.2 days/patient. Average of 2.1 CXR/patient. Average PIM score of patients - 33.5%. 79 of the xrays have been requested for urgent reasons (56%) whereas (44%) for routine actions. 47 of the urgent Xray were followed by a change in management (33.4% of total xrays and 55% of the urgent ones)

Conclusions: All our CXR have been requested based on clinical reasons and more than half of them had urgent indication. More than half of the urgent Xrays were followed by a change in management (1/3 of total). Although our study is not large, we conclude that CXR should be performed based on clinical indication only.

269

Critical / Emergency Care

The Effect of Simulation-Based Training Prior to Moving to a New Department on the Quality and Safety of Patient Care

Boris Fizdel ¹ , Evgeni Yakobuvski ¹ , Gideon Paret ¹ , Tal Sadeh Vered ¹ , Itai Pessach ¹ , Ortal Emergy ¹

¹ Pediatric ICU, Sheba-Academic Medical Center Hospital, Israel

Background: The PICU at Edmond and Lily Safra Hospital moved to its new 22-bed facility in February 2017. The transition included several profound changes in the department`s operating philosophy that affect daily work, such as closed patient rooms, the possibility of parents staying next to their child and replacement of all monitoring and warning systems while significantly increasing the area of the department. In the face of these extensive changes and the high risk to hospitalized patients during this transition period, a preparatory training program was prepared prior to the actual transition.

Method: Analysis of the challenges involved in the transition raised several key points: the need to test systems and work procedures, assimilate the changes in the new work routine, familiarize with new equipment and obtain the ability to provide adequate emergent critical care in the new work environment. In order to meet these challenges, an In-Situ simulation-based training program was created in the new department.

Results: Prior to the opening of the new department, the entire team underwent simulation-based training, which included 258 hours of training and testing of systems. Each simulation examined a different component of the daily work regime and management of extreme situations. Pitfalls were identified and re-examined in subsequent training.

Throughout the simulation-based training, more than 173 potential pitfalls were identified at different levels of severity, including 38 severe pitfalls that could endanger patient care.

Conclusions: Assessments and early simulation-based training in the new work environment allow early detection and correction of significant pitfalls and risks prior to moving to a new work environment. In addition, simulation reduces the level of anxiety and fear from transition, while increasing the responsiveness and readiness of the staff with a significant impact on the quality and safety of patient care.

666

Critical / Emergency Care

Role of Salivary Biomarkers in Pediatric Pain Assessment

Kristina Ganzijeva ¹ , Lina Jankauskaite ^1,2

¹ Medical Academy, Lithuanian University of Health Sciences, Lithuania ² Department of Pediatrics, Hospital of Lithuanian University of Health Sciences Kauno klinikos, Lithuania

Introduction: Pain self-reporting is currently a ‘golden-standard’ tool for pain assessment. However, it varies from patient to patient and could be inaccurately understood by healthcare professionals. According to several studies, acute as well as chronic pediatric pain remains misunderstood, under-diagnosed, and still under-treated. Moreover, accurate pain diagnostic markers are still missing.

Aims: To investigate salivary cortisol and melatonin concentrations in children with acute pain. To compare it with pain severity and vital signs changes.

Methods: We conducted pilot observational study in Kauno Klinikos Pediatric emergency department (PED). Twenty-six patients referred to PED due to acute pain were included into the study. We excluded patients with chronical conditions, under pain-killers or hormonal treatment. We recorded patient’s gender, age, vital signs (heart rate (HR), blood pressure (BP), respiratory rate (RR), temperature (t°) and oxygen saturation (SaO2)), pain characteristics (severity and duration of pain). Saliva samples were collected and stored in -80°C till cortisol and melatonin ELISA analysis.

Results: Sixteen boys and 10 girls were involved in our research. Age median was10 (4-16) years. Fourteen cases were trauma patients, 12 cases referred due to pain of other origin. we observed increased HR and BP due to pain. Other parameters were within age range. The median of cortisol and melatonin were 287.5(68-1330) and 17,6(8.6-46.8) pg/ml respectively. There was tendency of melatonin reduction with increased intensity of pain, however it was not significant (p=0.136). The longer the pain lasted, the higher cortisol levels were identified (p=0.01). No link was found between abnormal vital signs and changes in biomarkers.

Conclusion: Primary results show a cortisol rise with regard to pain duration. A drop in melatonin levels was observed with increasing pain. These results show cortisol and melatonin as potential biomarkers in acute pain diagnostics.

986

Critical / Emergency Care

A Silent Fate

Carolina Goncalves ¹ , Carolina Fernandes ¹ , Alexandra Andrade ¹ , Lucilia Aveiro ¹ , Francisco Silva ¹

¹ Pediatria, Hospital Dr. Nélio Mendonça, Portugal

Introduction: Carbon monoxide (CO) is an odorless, colorless, non-irritating, yet significantly toxic gas and is considered the leading cause of death by poisoning in the world. The clinical picture is variable and non-specific, ranging from mild to severe. Pediatric age range is a risk group, due to central nervous system immaturity and accelerated basal metabolism. In children, the clinical progression is more aggressive and difficult to detect than in adults.

Clinical Case: The following cases describe two siblings who were observed in a Pediatric Emergency Department.

Case 1:

A 10-year-old female, who after awakening suddenly from bitemporal headache, briefly lost consciousness but recovered within minutes. After recovering, she had an episode of vomiting. The patient’s physical examination was normal. ABG: pH 7.39, lact 1.4, COHb 25.2%.

Case 2:

A 7-year-old male, after waking to use the toilet, began having a headache and feeling dizzy with lipothymia, but recovered in minutes. The patient’s physical examination was normal. ABG: pH 7.37, lact 1.4, COHb 21.7%.

Both parents remained asymptomatic.

The diagnosis of CO intoxication was made, and the treatment protocol was fulfilled with oxygen therapy in a hyperbaric chamber. Two treatment sessions were performed, with the first treatment at 2 hours after hospital admission and the second occurring 12 hours later.Both patients were discharged 24 hours after the onset of the disease, with no associated symptomatology. Patients were instructed to follow up with a consultation with Hyperbaric Medicine at the 1st, 3rd and 6th month after the occurrence.

Conclusion: These two cases demonstrate the importance of clinical judgment to a rather linear history. Correct and timely diagnosis is essential, to ensure rapid action and minimization of possible associated complications. The coordination of the team and between several services is essential.

189

Critical / Emergency Care

Outcomes following Presentation with Concussion in the Paediatric Emergency Department: Missed Cases of Post-Concussion Syndrome

Laura Harrison ^1,2 , Lorcan Duane ^1,2

¹ Paediatric Emergency Department, Royal Manchester Children's Hospital, UK

² School of Medical Sciences, University of Manchester, UK

Background: 1 in 5 children will sustain a traumatic brain injury by the age of 16 years, with 90% of these being concussion. The majority recover to baseline function within 4 weeks, however 31% suffer from persisting symptoms. There is a lack of clear, consistent guidelines in the UK about management, discharge advice and follow-up of children with concussion. In our Paediatric Emergency department (PED) there was no follow-up arranged for any patients and no patient information leaflet.

Objectives: The aim was to identify any patients with concussion suffering from ongoing symptoms of post-concussion syndrome (PCS), who would benefit from follow-up. Additionals aims were to create an advice leaflet about concussion to be given to patients and make recommendations regarding systematic follow-up for future patients with concussion.

Methods: A search was conducted for patients aged 16 or under, discharged from the PED in the last 15 months, with a diagnosis concussion. Their medical notes were reviewed and a telephone survey of parents was conducted asking about current symptoms of PCS.

Results: The search yielded 121 patients. 68 of 121 parents of patients were able to be contacted. Of these, 33 out of 68 reported symptoms of PCS (48.53%) of new onset since their head injury, lasting longer than 4 weeks. School-aged children (5-12 years) were most commonly affected. The most common post-concussive symptoms reported were headaches, and problems with temper and impulsiveness.

Conclusion: Concussion management was found to be an area for improvement in this PED. A letter was written to the 33 children with PCS symptoms to arrange follow-up, and a new concussion leaflet for parents and patients was created to ensure follow-up was sought in the case of ongoing symptoms. Recommendations were made about the importance of starting systematic follow-up of all children with a diagnosis of concussion.

257

Critical / Emergency Care

Limp in Childhood: Are we Missing Significant Diagnoses?

Laura Harrison ¹ , Kate Hooper ¹ , Sahana Rao ¹

¹ Department of Paediatrics, Oxford University Hospitals, UK

Background: Limp is a common presentation and accounts for up to 5% of emergency department visits in children. It is difficult to clinically differentiate benign, self-limiting conditions like transient synovitis, from more serious conditions such as septic arthritis and osteomyelitis. It is therefore imperative to investigate and manage appropriately. Currently, there are no national guidelines for the investigation and management of limp in children in the UK.

Objective: The aim of this audit was to see how many children presenting with limp to the Children’s Clinical Decision Unit were being correctly investigated and managed according to local guidelines. Additional aims were to see if any significant diagnoses were missed and to update the guidelines as appropriate.

Methods: A search was done for patients presenting with limp to our Children’s Clinical Decision Unit, within the last 6 months. Their electronic medical records were then reviewed for details of their admission, investigations, diagnosis and any missed diagnoses. These were compared to local guidelines at Oxford University Hospitals Trust.

Results: The search found 106 patients. All patient should have had bloods and an x-ray, according to our guidelines. 33 of the 106 patients had the correct initial investigations. Those with 2 or more risk factors for septic arthritis should have had an ultrasound and this occurred in 10 of 15 patients. The most common diagnosis was transient synovitis (45 out of 106) and there were 2 cases of osteomyelitis and 1 case of septic arthritis. There were 4 missed diagnoses including 1 case of osteomyelitis.

Conclusion: Adherence to limp guidelines was poor and as a result significant pathology was missed. Recommendations included increased education of doctors about the limp management guidelines, creation of limp proforma and reinforcing mandatory telephone reviews after discharge. Further ongoing work includes reviewing and updating the limp guidelines.

720

Critical / Emergency Care

Paediatric Abdominal Trauma: Variety of Trauma Mechanisms, Resulting Injuries and Treatment

Melanie Kapapa ¹ , Katharina Esswein ¹ , Alexandre Serra ¹

¹ Department of Surgery University Hospital Ulm, Division of Pediatric Surgery, Germany

The study concentrated on data related to trauma mechanism in children (n=134) with blunt abdominal trauma like clinical findings, affected organs, results of radiologic imaging and clinical outcome. Points of interest were patient´s age and sex, time and season of accident, mechanism of injury, involved objects, severity of injuries, treatment and complications. Accident mechanisms varied depending on age and male patients had higher severe grades of injury pattern (p=0.008). Boys are more often (in 67,9%) involved to suffer an intraabdominal injury (IAI) with severe grades of injury pattern (p=0.014). Depending on age, proportions of various accident mechanisms occurred. Leading trauma was in 56% a fall, most common less than 1meter, followed by sport related trauma (41,8%) and road traffic accidents (21.6%). In children with abnormal laboratory studies the grade injury pattern was more severe (p0,001). Focused Assessment with Sonography for Trauma (FAST) was primary performed in 85,8% and correlation between free abdominal fluid and injury pattern was significant (p0.001). 41,8% had an IAI with organ laceration and 46,3% had additionally injuries like head or thorax trauma. Conservative treatment of IAI was performed in 60,7% (n=34) and invasive treatment with abdominal surgery in 39,2% (n=22). Complications occurred in 34.4%, significantly after spleen (p=0.001), liver (p=0,001) and GI-tract injuries (p=0,001). Hospital duration stay increased with severity of injury (p0.001), especially after child abuse. Despite rarity of massive IAI after child abuse, it is vital for the affected children that involved medical staff kept this potential trauma issue in mind. We showed that risks for suffering severe IAI are present even during everyday activities, not just in traffic accidents. Focus should be on prevention on risk children, adapted to their age and danger potential. Parents have to be enlightened about risks directly and should train their children to perceive them.

816

Critical / Emergency Care

E-Bike-Related Cranial Injuries in Pediatric Population

Yevgeny Karepov ¹ , Danil A. Kozyrev ¹ , Moni Benifla ² , Vladimir Shapira ² , Shlomi Constantini ¹ , Jonathan Roth ¹

¹ Department of Pediatric Neurosurgery, Department of Pediatric Neurosurgery, Dana Children’s Hospital, Tel-Aviv Medical Center, Tel-Aviv University, Israel ² Pediatric Neurosurgery Unit, Pediatric Neurosurgery Unit, Rambam Health Care Campus, Haifa, Israel, Israel

Purpose: E-bikes are being used increasingly by all age groups. Children riding e-bikes often do not use safety equipment such as helmets, and are at increased risk for injuries requiring neurosurgery. The most common type of injury among pediatric e-bikers is head and neck trauma. We describe our experience treating cranial injuries.

Methods: Data regarding children (18 years old) with e-bike-related cranial injuries were collected retrospectively from two tertiary centers.

Results: Twenty patients were included. Seventeen were e-bike users, and three were hit as pedestrians. The average age at admission was 11.3 ± 4.85 (range 1.5–17) years old. All 17 e-bike users did not wear a helmet. Seventeen of the 20 (85%) suffered from skull fractures (70% involving the frontal bone), nine involving more than one region. Six patients (30%) had intracerebral contusions, 3 (15%) an epidural hematoma, and 6 (30%) a subdural hematoma. Three patients (15%) underwent surgery, two of them for depressed skull fracture reduction, and one for insertion of intracranial pressure monitor. One patient died (5%); 1 (5%) had a Glasgow Outcome Scale (GOS) of 3, 5 (25%) had a GOS of 2, and 13 (65%) were discharged without any neurological deficit (GOS 1).

Conclusion: E-bikes may inflict various cranial injuries, including fractures and intracranial bleeds, and may lead to significant morbidity and mortality. Education of children to use protective gear, wide exposure of younger adolescents to traffic laws, and limiting the use of e-bikes to older children, are all necessary actions.

711

Critical / Emergency Care

Predictors of Caregiver Satisfaction in Pediatric Laceration Repair in Pediatric Emergency Department

Soyun Hwang ¹ , Hayoung Kim ¹ , Jin Hee Lee ² , Young Ho Kwak ¹ , Do Kyun Kim ¹ , Jae Yun Jung ¹ , Hyuksool Kwon ² , Dongbum Suh ² , Yoo Jin Choi ² , Joong Wan Park ¹ , Se Uk Lee ¹ , Ik Chang Choi ¹

¹ Emergency Medicine, Seoul National University Hospital, South Korea ² Emergency Medicine, Seoul National University Bundang Hospital, South Korea

Introduction: Laceration repair is one of the most common procedures performed in pediatric emergency department (PED). The objective of this study was to define the factors of care that are important to caregivers during a pediatric laceration repair and overall ED satisfaction.

Methods: This was a cross-sectional observation study performed in an urban, tertiary hospital. A convenience sample of patients younger than 18 years old presenting to PED for laceration repair was recognized and their caregivers were enrolled to complete a survey developed for this study. Demographic data of patient and caregivers were analyzed. Univariate and multivariate logistic regressions were used to determine which of these factors are related to satisfaction in the process of laceration repair and the overall ED process.

Results: Total 55 caregivers were enrolled. 39(70.9%) of the caregivers were fathers. Majority of children had facial laceration (n=44, 80%) and median ED length of stay was 181 (IQR, 157-208) minutes. the median age of the children was 41.8 (IQR, 23-91) months and the median age of the caregivers were 37 years old (IQR, 35-41). Most of the laceration was repaired by plastic surgeons (81.8%). In the multivariate regression analysis, ‘preparing before procedure’, ‘mid-income family’, ‘caring attitude of nurse’, ‘cosmetic outcome’, and ‘measures to control patient’s anxiety’ were significantly related to the caregiver’s satisfaction about the laceration repair (p0.05), while ‘preparing before procedure’ and ‘ED environment’ were significantly related to the caregiver’s satisfaction about the overall ED process (p0.05).

Conclusions: ‘Preparing before procedure’ was significantly related to the caregiver’s satisfaction in both pediatric laceration repair and overall PED experience. The strongest predictors were ‘cosmetic outcome’ for laceration repair process and ‘prepare for the procedure’ for overall PED experience. Our findings suggest that improvements in various aspects will result in more satisfied parents.

528

Critical / Emergency Care

Apnoeic Event in Wolf-Hirschhorn Syndrome: Could this have been a “Tet” Spell?

Kene Maduemem ¹ , Gheetha Arumugam ¹ , Amol Chingale ¹

¹ Paediatrics, Lincoln County Hospital, UK

Background: Wolf-Hirschhorn syndrome (WHS) is a rare genetic condition caused by partial deletion of short arm of chromosome 4. Clinical manifestations comprise but not limited to typical facie, mental retardation, growth delay, epilepsy, and congenital cardiac defects. Congenital heart defects occur in about 25-50% of cases.

Objective: We describe a child with WHS presenting with a femoral fracture who went apnoeic in the course of management resulting in a cascade of events.

Methods: Clinical presentation, laboratory and radiological findings are discussed.

Results: A 4.5-year-old boy known to have WHS was admitted with left femoral fracture. The syndrome was an antenatal diagnosis. Other associations noted were severe growth delay (weight of 6kg), epilepsy, global developmental delay, visual impairment, and tetralogy of Fallot (TOF). His TOF has been managed uneventfully on propranolol with acceptable oxygen saturation (Sp02) in the 70s. The spontaneous fracture required Gallows traction, which provoked distress. He developed hypoxic crisis requiring oxygen therapy to maintain acceptable SpO2. Subsequently, he went apnoeic and dusky needing bag and mask ventilation. Blood gas revealed severe metabolic acidosis, which improved after fluid bolus. Knee-chest position could not be attempted due to the fracture. Although he got treated for suspected sepsis due to the dilemma of events, he did have a hypercyanotic spell in hindsight. He was subsequently discharged with conservative management of the fracture.

Conclusion: To the best of our knowledge, this is the first reported case of hypercyanotic (Tet) spell in WHS. This rather uncommon event resulted in a significant learning point for medical and nursing staff.

319

Critical / Emergency Care

“Small Things” make a Huge Difference in the Emergency Department

Kene Maduemem ¹ , Muhammad Adil Mughal ¹

¹ Paediatrics, Lincoln County Hospital, UK

Background: The assessment of children in a busy mixed adult and paediatric emergency department (ED) can be challenging. Good support from the ED staff not only improves patient safety but also, renders care delivery less daunting.

Objective: To survey the clinical experiences of junior doctors in the resuscitation room and establish probable factors affecting patient safety before and after implementation of key recommendations.

Methods: An initial survey on the experiences of junior doctors during assessment of children in resus bays was conducted in October 2018. Some recommendations deduced were implemented; provision of sepsis trolley and grab box. A repeat survey in January 2019 was done to evaluate impact of implemented changes.

Results: Twenty doctors participated in the initial survey while 19 in the later survey. First survey was designated T1 and the repeat survey T2. All respondents reported attending at least 2 resuscitations in both surveys. Ten percent in T1 vs. 35% in T2 responded that a nurse was always present during resuscitation events. Ninety percent had performed or assisted in at least 1 procedure in the resuscitation bay in both surveys. Using a 5-point Likert scale, 40% of respondents in T1 vs. 63% in T2 were satisfied with the level of support of ED staff. Easy access to materials for resuscitation was rated good by 10% in T1 vs. 81% in T2. Over 70% in either survey requested resuscitation drugs; promptness of drug retrieval and administration was rated good by 13% (T1) vs. 45% (T2). The grab box was used by 65% in T2 with excellent feedbacks on its fall-back benefit.

Conclusion: Significant improvement was noted in care delivery following the implementation of the new sepsis trolley and grab box. There still exist areas for continued development in enhancing safe care delivery in this mixed adult and children ED.

902

Critical / Emergency Care

Post-extubation Acute Pulmonary Oedema in an Adolescent

Catarina Amaro ¹ , Joana Mendes ¹ , Pedro Protázio ² , Carlos Rodrigues ¹ , Sofia Ferreira ¹

¹ Paediatrics Department, Centro Hospitalar Universitário Cova da Beira, Portugal

² Anaesthesiology Department, Centro Hospitalar Universitário Cova da Beira, Portugal

Background: Post-extubation Acute Pulmonary Oedema (PEAPO) occurs in 0.1% of patients undergoing general anaesthesia, mostly in healthy children and young individuals, usually immediately after tracheal extubation. In paediatric age it has been rarely described, but the actual incidence is thought to be underestimated as many cases of post-operative respiratory distress may be misdiagnosed.

Case report: A former healthy 15-year-old male was admitted to the emergency department with symptoms and physical examination compatible with acute appendicitis. A laparoscopic appendectomy was performed under general anaesthesia. Immediately after extubation, during which neither aspiration nor vomit were observed, he developed hypoxaemia (SpO₂ 82%), intense coughing and polypnea, requiring additional O₂ (FiO₂ 40% using Venturi mask). He was hemodynamically stable, with normal cardiac auscultation and without oedema. His thorax expansion was symmetrical, and pulmonary auscultation presented symmetrical decreased breath sounds with dispersed fine crackles. Arterial gasometry showed pH 7.36, pCO₂ 45mmHg, pO₂ 48mmHg, HCO₃^- 26.8mmol/L, BE -0.5mmol/L, and the chest radiograph revealed bilateral diffuse opacities with ill-defined edges, suggesting acute pulmonary oedema. Intravenous furosemide was administered, and a quick recovery was noticed. The drug was maintained during 3 days, as well as supplementary oxygen, with progressive decreasing. With favourable evolution, the patient was discharged 4 days after surgery, asymptomatic, with normal cardiac evaluation and chest radiograph.

Conclusion: Although rare, PEAPO is a potentially fatal complication, which may worsen the prognosis of low-risk surgical procedures. It is important to recall this diagnosis in children and adolescents who present signs of respiratory distress and hypoxia after general anaesthesia, as prompt intervention is essential for a favourable outcome.

798

Critical / Emergency Care

The Introduction of Paediatric Cases to Pre-Hospital Grand Rounds

Ronan Callanan ^1,2 , Kasia Domanska ¹ , Anne-Marie Murphy ² , Alan Watts ¹

¹ Department of Emergency Medicine, University Hospital Limerick, Ireland

² Department of Paediatrics, University Hospital Limerick, Ireland

Background: Paediatric emergencies can be challenging and intimidating for providers in the pre-hospital setting. Pre-hospital practitioners require a skill set very different to interventions performed in the adult population. These include challenging intravenous access, medication calculation, potential external stressors and a variety of clinical conditions seen less frequently.

Methodology: Pre-hospital Grand Rounds (PHGR) is a relatively new concept. It was introduced in St Vincent’s University Hospital Dublin with the programme in University Hospital Limerick (UHL) commencing in October 2018. Following discussion with pre-hospital colleagues in the midwest region the coordinators of PHGR UHL acknowledged the importance and potential benefit of inclusion of paediatric cases at each session. A survey was conducted to gauge pre-hospital provider views on PHGR UHL as a source of paediatric emergency education.

Discussion: At each meeting a case is presented by a paramedic or advanced paramedic. This includes assessments, interventions on scene and subsequent handover in the Emergency Department. An Emergency Department doctor subsequently discusses the management of the child and outcome. A brief teaching session related to the primary diagnosis follows in an open forum with the audience, consisting of pre-hospital providers, nursing and medical staff. The meeting is supervised by a Consultant in Emergency Medicine with input from the Department of Paediatrics and National Ambulance Service training officers.

When surveyed, participants considered PHGR UHL as an educational resource for paediatric emergencies, considered the meeting to add to their practice and found paediatric emergencies posed different challenges to the adult population.

Conclusion: The introduction of paediatric cases at PHGR UHL has been met with enthusiasm and in its infancy is being considered a beneficial resource. It creates a link of communication between pre-hospital clinicians and hospital providers. The meetings do not follow a template but are developed through feedback and communication between all parties.

836

Critical / Emergency Care

Transient Loss of Consciousness Resulting in Syncope: Review of International Clinical Practice Guidelines

Leona Nertney ^1,2 , Kimran Sidhu ¹ , Paul McNally ^1,2

¹ Department of Paediatrics, Royal College of Surgeons in Ireland, Ireland ² NCRC, National Children's Research Centre, Ireland

Background: Syncope is a symptom characterised by transient loss of consciousness due to global cerebral hypoperfusion. 30-50% of children and adolescents experience a faint. Syncope results in 1-3% of all visits to the Paediatric Emergency Department. While benign pathology dominates, recurrent events, ancillary injuries or anxiety around sinister pathology can bias clinical judgement. Resulting heterogeneity in clinical care drives wasteful investigations or referrals to oversubscribed services. Here we review state-of-the-art International Clinical Practice Guidelines (CPG) which may be used to reduce disease burden.

Methods: Structured review of Cochrane, Pubmed and Web of Science databases performed using search terms: (“Clinical Practice Guideline” AND “Paediatric” (OR “Pediatric”) AND “Syncope”), (“Patient Algorithm” OR “Diagnostic Algorithm” AND “Paediatric” AND “Syncope”).

Results: Our search generated 126 articles, with further review yielding five CPG’s: European Society of Cardiology²⁰¹⁸, American Heart Association²⁰¹⁷, Canadian Cardiovascular Society/Pediatric Cardiology Association²⁰¹⁷, NICE²⁰¹¹, Chinese Pediatric Cardiology Society²⁰¹⁸. Guidelines used “Class I Evidence” or “Strong Recommendation” to support the role of the following: History (peri-event features), Physical Examination plus Family History (Epilepsy, premature cardiac death in 1^st/2^nd degree relative 40yrs). All but one endorsed ECG as a necessary diagnostic test. In the absence of positive findings, no further investigation is required. While presence of symptoms related to exertion should receive diagnostic work-up with referral to specialist services e.g. syncope unit, cardiology. Reassurance, education/ lifestyle measures remain the cornerstone of clinical management. Guidelines expanded on this to include medication use for refractory cases, maintaining psychological wellbeing and autonomic function training.

Discussion: Adherence to evidence-based guidelines ensures clinically appropriate management of syncope, whilst reducing wasteful investigations or referrals. Although all guidelines reviewed agree on history and examination, specific differences around ECG use, or non-pharmacological treatment exist.

743

Critical / Emergency Care

Procalcitonin Useful Diagnostic Marker for Treatment the Asphyxed Preterm Newborns with Sepsis

Danilo Nonkulovski ¹ , Aspazija Sofijanova ¹ , Silvana Naunova Timovska ¹ , Mica Kimovska ¹ , Tamara Voinovska ¹ , Hristina Mangzukovska ¹ , Spasija Neskova ¹ , Olivera Jordanova ² , Aleksandra Janchevska ³ , Vankuver Mancev ⁴

¹ Neonatal intensive care unit,, University Children Hospital, Macedonia

² Clinical laboratory, University Children Hospital, Macedonia

³ Endocrinology, University Children Hospital, Macedonia

⁴ Pediatric, Hospital, Macedonia

Background: Birth Asphyxia can lead to severe hypoxic ischaemic organ damage in newborns followed by neurodegenerative diseases, mental retardation and epilepsies, leading to significant mortality and morbidity in the Intensive Care Unit at the University Children Hospital-Skopje.

Objective: The aim of this study was to determining the corelation between the levels of PCT levels in pretern newborns with asphyxia and term newborns with asphyxia, with sepsis and proven blood culture

Methods: In this study we include the 31 (M:F=14:17) sepsis newborns with asphyxia and proven blood culture. They have been divided into two groups I group included 11 septic preterm newborns with asphyxia and II group - 20 septic term newborns.. Results of WBC, C-reactive protein (CRP) and PCT, were recorded. Procalcitonin levels was assessed before the beginning of the antibiotic treatment and second measurement after 3 days, by using a immunoassay system Vidas based on the Enzyme Linked Fluorescent Assay (ELFA) principles.

Results: We confirmed Staphylococcus , methicillin-resistance gene in all preterm newborns. 14 term newborns were with confirmed Staphylococcus , methicillin-resistance gene and in other 6 term newborns were confirmed Staphylococcus and Streptococcus pneumonia. The values of procalcitonin (PCT) were considerably increased in septic preterm newborns with asphyxia, relative to term newborns. This difference was statistically significant. The values of C-reactive protein gradually increase after 24-36 hours in both groups. The second measurement of PCT after 3 days is a parameter, who show us whether an appropriate antibiotic for the treatment is used and may prevent severe sepsis and septic shock in newborns.

Conclusion: Increased PCT level was significantly associated with preterm newborns with confirmed Staphylococcus , methicillin-resistance gene. The value of PCT is a reliable parameter whether an appropriate antibiotic treatment is used in newborns with asphyxia, and and gram positive blood culture

918

Critical / Emergency Care

Factors Associated with Increased Risk for Pediatric Orbital Cellulitis – Who should we scan?

Lea Ohana Sarna Cahan ¹ , Noa Hurvitz ² , Itai Gross ³ , Adi Cohen ² , Saar Hashavya ³

¹ Department of Pediatrics, Hadassah-Hebrew University Medical Center, Jerusalem

² Faculty of Medicine, Hadassah-Hebrew University Medical Center

³ Department of Pediatric Emergency Medicine, Hadassah-Hebrew University Medical Center, Jerusalem

Background: Periorbital cellulitis (POC) is a common infection in the pediatric population with minor complications. Orbital cellulitis (OC) carries Potential devastating complications of intracranial infection and vision impairment. Precise evaluation of a child with POC\OC is complicated due difficulties in physical examination and risks of imaging by CT.

Method: A retrospective review of children aged 0-16 years admitted to the ED for POC or OC from 2009 to 2019.

Results: Over the ten years study period, 247 children 16 years were admitted to the ED with the diagnosis of OC\POC. Mean age 5.2 ± 4.5 years of age.

OC documented in 50 patients. The mean age was 7.8 ± 4.3 years. Fever (79.6%), URTI (42.9%), swelling of both eyelid (98%), proptosis (30.6%), and tenderness on percussion (24.5%) were all more common in comparison to POC with P values of 0.0001, 0.03, 0.0001, 0.0001,0.0001 respectively. All children with diagnosis of OC underwent CT scan. Abscess was documented in 63.3% of the OC patients.

POC accounted for 247 patients. Mean age was 4.6 ± 4.3 years. In 19.8% of the cases local trauma or bite mark in the infected eye were recorded.

Mean leukocyte count (10⁹\L) in OC group 15.2 vs 13.4 (P= 0.05), ANC was significantly higher in the OC 11.3 vs 7.2 (P=0.0001) whereas the lymphocyte count was higher in the POC 4.5 vs 2.4 (P= 0.0001). Laboratory inflammation signs in OC had mean CRP levels (MG%) of 11.7 vs 4.9 (P =0.0001) and ESR of 53.6 vs 36.4 (P= 0.02).

Conclusion: Differentiation between OC an POC is cardinal. while previously findings on clinical examination such as ophthalmoplegia and proptosis were found indicative of OC rather than POC, our study highlights the importance of inflammatory markers including ANC, CRP and ESR in the assessment of infections of the eye.

959

Critical / Emergency Care

Metamizol Sodic - Milliliters or Drops ?

Carmen Olaru ¹ , Valentin Olaru ² , Tamara Solange Roșu ¹ , Ileana Katerina Ioniuc ³ , Smaranda Diaconescu ³

¹ Emergency Room, Emergency Children Hospital ,,Sf. Maria", Romania ² Intensive Care, Emergency Children Hospital ,,Sf. Maria", Romania ³ Pediatrics, Emergency Children Hospital ,,Sf. Maria", Romania

Background: Accidental poisoning with metamizol sodic, by confounding the number of drops with the number of milliliters are going to become an important problem of admission in our emergency department.

Objectives: The aim of this study was to describe signs and symptoms of the intoxication, the evolution, the treatment and to established some measures to avoid these situations.

Material and Methods: A retrospective study was performed. The study group includes 12 children admitted in the emergency room due to January 2017 until December 2018.

Results and Discussions: In the study group, age between 7 months and 8 years, the children received a number of milliliters of metamizol sodic equal with the number of drops necessary. Time between ingestion and the admission in the emergency room varied between a few minutes (when the parents took note the incident), until 72 hours. The maximum dose was 15 grams. The symptomatology was severe (seizures, sleepiness) in 3 cases, moderate (vomiting, nausea, abdominal pain, purpura) in 6 cases, 3 cases were asymptomatics. Laboratory investigations revealed leucopenia in 5 cases, trombocitopenia-1, hepatocytolisis - 3, modifications of renal function tests- 3, and in 3 were cases normal. The treatment consisted in gastric lavage- 4 cases, forced diuresis, hepatoprotectives, corticosteroid therapy - 3 cases. The outcome was favorable in all the cases, 3 children developed cutaneous rash and 2 bronchospasm after few days.

Conclusion: Be careful with metamizol sodic dosage!

291

Critical / Emergency Care

Button Battery Ingestion-Case Series from Lakonia, Greece

Aikaterini Pana ¹ , Olympia-Panagiota Rozakea ¹ , Maria-Maroudia Berikopoulou ¹ , Panagiotis Panas ² , Sofia Vasilakou ¹ , Afroditi Barmpakou ¹ , Ioannis Panagiotou ¹

¹ Pediatrics, General Hospital of Lakonia- Nursing Unit of Sparta, Greece

² Obstetrics-Gynaecology, University Hospital Of Patras "Panagia i Voitheia", Greece

Objectives and Study: Button battery ingestions epitomize the challenge among pediatric foreign body ingestions as the outcome can vary from harmless to lethal. The serious outcomes refer to children younger than 5years old and when ingested batteries are larger than 20mm-diameter(higher-risk patients).

Esophagus is the target tissue of caustic injury and acute endoscopic treatment is needed. In cases, where battery lies beyond the esophagus (stomach, bowel etc) and the patient is asymptomatic, abdominal radiographs and observation are advocated. Endoscopic removal is suggested only if the battery remains inside the stomach 4 days after ingestion.

Methods: We report two cases of toddlers, presented in our pediatric department, that ingested button batteries with their diagnostic X-rays.

The first was a 3-year-old girl that ingested a battery smaller than 12mm-diameter (10mm) 2^1/2 hours prior to her admission. Her vital signs were stable (HR:108-114/min) and was asymptomatic. An X-ray was obtained that revealed a radiopaque object(battery) in the bowel. From the laboratory exams, only increased amylase levels were found(221U/l, with normal values:30-118U/l).

The second was a 2.5-year-old girl that ingested a battery larger than 12mm-diameter ( 20mm) 1 hour prior to her admission. Her vital signs where stable (HR:100/min) and also remained asymptomatic. The X-ray that was performed revealed a battery in the stomach. No laboratory exams were obtained.

Results: Both children received intravenous(iv) fluids and ranitidine as prophylaxis and were immediately transferred to a pediatric hospital with endoscopic unit. They remained asymptomatic throughout their stay and endoscopic intervention was not needed. The battery was dismissed through the stool in both cases, during the second( 2^nd) and third( 3^rd) day after ingestion, respectively.

Conclusion: Button battery ingestions, where battery lies beyond the esophagus and the patient remains asymptomatic, should be managed with caution, as esophageal damage cannot be excluded. Endoscopic intervention should be within immediate reach whether or not it is actually performed in the end.

109

Critical / Emergency Care

Prevalence and Outcomes of CPR in Pediatric In-Hospital Cardiac Arrest: A 7-year Experience at a Single Center in Thailand

Uthen Pandee ¹ , Thita Pacharapakornpong ¹ , Jarin Vaewpanich ¹ , Nattachai Anantasit ¹

¹ Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Thailand

Background: Children with in-hospital cardiac arrest (IHCA) has variety outcome in difference centers and country.

Objective: The objective in this study was to report the prevalence, characteristic and outcomes of IHCA and also evaluate associated factors in survival.

Methods: A retrospective study including children aged 1 month to 18 years were diagnosed with cardiac arrest and received cardiopulmonary resuscitation between January 2010 and December 2016. Descriptive and logistic regressions analysis was used to analyze factors that related to the return of spontaneous circulation (ROSC) and survival.

Results: Total of admission was 32,122. Of 88 patients (0.27%) were enrolled. Median age of patients was 1.21. The first documented rhythm was pulseless electrical activity (43%). The overall sustained ROSC and survival rate were 62.5% and 15.9%, respectively. Of 78% of those who survived had favorable neurological outcome. The most common cause of IHCA was septic shock. Ischemic cause, development of subsequent asystole and receiving CPR more than 20 minutes had significant higher sustained ROSC rate (p0.05). Multivariate analysis to adjust for the clinical variables significantly associated with failure ROSC confirmed that patients with prolonged duration of cardiopulmonary resuscitation (CPR) and subsequent asystole had higher failure ROSC (p0.05). However, patients who received vasoactive medications less than two drugs had higher hospital survival rate (p=0.024).

Conclusion: Duration of CPR was the most important factor associated with achieved ROSC, whereas number of vasoactive medications was the factor associated with hospital survival rate.

738

Critical / Emergency Care

Implementation of the Paediatric Early Warning Score in the Observation Room of the Emergency Department of a Second Level Hospital

Miguel Paiva Pereira ¹ , Hugo de Castro Faria ¹ , Paula Poção ¹ , Ana Serrão Neto ¹

¹ Centro da Criança e do Adolescente, Hospital CUF Descobertas, Portugal

The Paediatric Early Warning Score (PEWS) was initially developed to pre-emptively identify hospitalized children who were at risk of clinical deterioration^1,2. A study in a paediatric Emergency Department (ED) found that an elevated score was associated with need for Intensive Care Unit (ICU) admission³. A lot of PEWS systems have been developed but a recent systematic review noticed lack of consensus on which PEWS is most effective, although improved clinical outcomes were reported⁴. In July 2017 we implemented the Irish PEWS⁵ in our paediatric ED aiming to objectify and improve clinical monitoring in our unit.

The purpose of this study is to review our data by verifying if a higher PEWS score correlated to clinical deterioration and if it is applicable to our clinical practice.

To do so we evaluated medical records of patients who were applied the PEWS between July 2017 and March 2019 and registered the maximum score (MS). We defined clinical deterioration as the need for ICU transfer, higher respiratory support or the presence of sepsis criteria.

We evaluated 840 children admitted to the ED observation room, whose age and MS distributions are represented, respectively by the graphics 1 and 2. The MS was ≥4 in 181 (22%). For a MS ≥4, the sensibility and specificity for clinical deterioration was respectively 96.5% and 84.0%. Respiratory diseases were the most common diagnosis (227 patients), with 135 (59%) MS ≥4, whose sensibility and specificity for clinical deterioration were, respectively, 100% and 48.9%. We also evaluated this score for MS of 5 and 6.

In addition of being a system that allows a standardized surveillance, the use of this score seems to improve patient safety and medical decision, particularly by excluding clinical deterioration, as showed by the high sensitivity values reached using the MS of 4 as a cut-off.

405

Critical / Emergency Care

Sedation and Analgesia in a Pediatric Intensive Care Unit (PICU): Point of Care Study

Andrzej Piotrowski ^1,2,3 , Olga Stańkowska ^1,2,3 , Adam Grabowski ^1,2,3 , Andrei Trebukhousky ^1,2,3 , Jolanta Gardocka ^1,2,3

¹ Anaesthesia and Intensive Care, Children's Memorial Health Institute, Poland ² Anaesthesia and Intensive Care, Children's Memorial Health Institute, Poland ³ Anaesthesia and Intensive Care, Children's Memorial Health Institute, Poland

Background: Variety of sedation methods are applied for children treated in the PICU without clear evidence about their usefulness. Opioids and benzodiazepines have been a standard analgesic and sedative combination. Currently newer agents, like a2-adrenergic agonists have been proposed.

Objective: To assess the principal sedative and analgesic drug choices for patients treated in a pediatric medical/surgical ICU.

Methods: The study was conducted in a 24 bed complex of two intensive care units, surgical and medical, at one institution. A one day analysis was chosen. The medications, their methods of administration, and doses were analyzed.

Results: There were 24 patients treated in both wards on the day of study, 20 of them required mechanical respiratory support. The majority of patients (13) were up to 3 days after surgery. The most common analgesic drug was morphine used in 16 children (in 9 as bolus, in 5 as infusion) administered for a median of 2 days (range 1-7), followed by non-opioid drugs (paracetamol and metamizole) in 6 cases, and sufentanil by infusion in 2. The major medication for sedation was midazolam (14 patients, in 5 as an infusion) administered for a median of 3 days (range 1-22), followed by dexmedetomidine infusion in 9 patients (median duration 4 days, range 1-22 days, mean 8 days). The doses of morphine were in the lower therapeutic range (mostly 0,1 mg/kg bolus), doses of dexmedetomidine were from 0,6 to 1,5 micrograms/kg/hr. Oral methadone was administered in 2 children, in one in combination with clonidine. Ten patients were treated concomitantly with 2 analgesic/sedative drugs, five 3 medications, four were receiving 4 drugs, and five one.

Conclusion: In our study morphine, midazolam, dexmedetomidine were main analgesic and sedative drugs used in the PICU. Dexmedetomidine was used for longer periods of time than suggested by the manufacturer and literature.

469

Critical / Emergency Care

Familial Hypokalemic Periodic Paralysis

Irina Rosa ¹ , Bernardo Camacho ¹ , Paulo Sousa ² , Francisco Silva ¹

¹ Pediatria, Hospital Dr Nélio Mendonça, Portugal ² Neuropediatria, Hospital Dr Nélio Mendonça, Portugal

Introduction: Periodic Paralysis (PP) is a rare neuromuscular disease related to a defect in muscle ion channels, characterized by sporadic episodes of painless muscle weakness that can be precipitated by vigorous exercise, prolonged fasting, or carbohydrate-rich meals. PP is classified as hypokalemic when episodes occur associated with low levels of potassium in the blood. The crises start in late childhood or early adolescence and vary in frequency and duration. Treatment during the acute phase is administration of potassium chloride.

Case Report: A 12-year-old female adolescent appealed to the emergency department due to sudden onset of tetraparesis. She had no difficulty breathing nor signs of respiratory distress. Cardiac, pulmonary and abdomen examination was normal. Conscious and oriented in time and space. Neurological examination showed grade II muscle strength in the lower limbs and grade III in the upper limbs. Osteotendinous reflexes were absent. Facial mimic was present.

Upon this presentation the mother promptly revealed a familiar history of periodic hypokalemic paralysis affecting several members.

Blood analysis showed an isolated severe hypokalemia of 1,7-2,0 mmol/L. ECG showed prolonged PR interval, mild ST depression, prominent U waves.

Slow correction of potassium was promptly started with gradual recovery of muscle strength and ECG stabilization. She was discharged 20 hours after therapy onset and medicated at home with daily acetazolamide and potassium chloride if onset of symptoms. Genetic study was started for índex familial mutations.

Conclusion: Periodic familial paralyzes are rare conditions, the most frequent being the hypokalemic form. The diagnosis of PP can be confirmed by genetic tests, which are recommended as first line when there is an intermediate to high degree of clinical suspicion. This case report is intended to emphasize the importance of the clinical history and a complete physical examination on diagnosis. Treatment should not be delayed. Genetic family counseling should be offered.

631

Critical / Emergency Care

Alkaline Lemonade Poisoning: A Case Report

Virginia Santana Rojo ¹ , Marta Bautista Barea ¹ , Mariano Silva Hernández ¹ , José Manuel Caballero Caballero ¹ , Isabel González Bertolín ² , Paula García Sánchez ² , Rosario López López ² , Francisco Javier Aracil Santos ¹

¹ Department of General Paediatrics and Paediatric Infectious Diseases, Hospital Universitario La Paz, Spain ² Paediatric Emergency Department, Hospital Universitario La Paz, Spain

Background: First-line treatment for acute gastroenteritis (AGE) are oral rehydration solutions. These prepared solutions are recommended by WHO and ESPGHAN since they assure a right replacement of hydro-electrolitic disbalance, advising against other alternatives.

‘Alkaline lemonade’ is a home-made alternative to prepared solutions, made with water, sugar, salt, lemon and baking soda. It has been widely recommended and extensively used in Spain due to its availability and effectiveness. We report a case of metabolic alkalosis and electrolytic disturbances in a patient with AGE, who was treated with 'alkaline lemonade'.

Case Presentation: A previously healthy 11-month-old boy was brought to the Emergency Department with an AGE caused by Norovirus. On his arrival he was afebrile and hemodynamically stable, with signs of moderate dehydration. Admission blood tests revealed metabolic alkalosis (pH 7.54, pCO2 45.7 mmHg and bicarbonate 38.4 mmol/L), hypokalemia (2.3 mmol/L) and hypernatremia (145 mmol/L), with normochloremia, were observed in first blood tests. The electrocardiogram showed flat T-waves in all derivations, without any other changes. He was admitted to the Paediatric Intensive Care Unit, where a fast correction of hypokalemia was performed, with disappearance of electrocardiogram disturbances. He received intravenous fluid therapy until complete recovery of detected alterations.

Due to the importance of disturbances, tubulopathies were ruled out. When the parents were asked about their son’s home management, they reported having administered home-made ‘alkaline lemonade’, without being able to specify the amount of baking soda, but bigger than the recommended ‘point of knife’ quantity. Therefore, the ‘alkaline lemonade’ poisoning along with the dehydration caused by AGE, explain the magnitude of ionic and acid-base disorders.

Conclusions: 'Alkaline lemonade' recipe is not very accurate, which can lead to elaboration mistakes, as our patient´s case. In conclusion, we should recommend oral rehydration solutions for AGE management and inform appropriately about their alternatives.

746

Critical / Emergency Care

Falls Under 6 Months of Age: 7-year Period at a Portuguese Hospital

Ana Sofia Vilardouro ^1,3 , Joana de Brito Chagas ^2,3 , Tânia Carvalho ³ , David Marques Lito ³

¹ Service of Paediatrics, Department of Paediatrics, Santa Maria Hospital - North Lisbon Hospital and Universitary Centre, EPE, Portugal ² Department of Paediatrics, Paediatric Hospital, Coimbra Hospital and Universitary Centre, EPE, Portugal ³ Service of Paediatrics, Vila Franca de Xira Hospital, Portugal

Background: Falls are the third leading cause of death by unintentional injury in European Union children and represent an important cause of incapacity. There is lack of scientific research on the less-than-6-month-old group.

Objective: To describe epidemiologic characteristics, outcomes and factors related to a worse outcome of falls in a specific age group children.

Methods: Retrospective descriptive study of all infants younger than 6 months admitted to our Emergency Room (ER) because of unintentional fall. The study was conducted at a second level Portuguese hospital over a 7-year period (2012 through 2018).

Results: There were 112 ER admissions related with falls in this group (0,045% of all admissions).

Forty-eight percent had fallen from baby equipment. The majority (82,4%) occurred from a 50-100cm high and head trauma was present in 94,8%. Symptoms occurred in 23,2% of infants and head injury was the most common finding on medical examination (47,3%). Serious injuries were seen in 16,1%. Imaging abnormalities were identified in 7,1% (all with head fracture; one third with brain injury) and 6,3% required hospital admission or transference to another hospital.

Serious injury was more frequent in infants younger than 3 months (66,7% vs 33,3%, p=0,009), in those who had fallen from more than 76cm high (p=0,013) and when medical examination revealed alterations (72,2% vs 27,8%, p=0,032). Radiography with suspected skull fracture was significantly associated with fracture on computed tomography (p=0,033). No deaths or permanent sequelae were found.

Conclusion: In our study the most serious injuries occurred in infants younger than 3 months and from relatively small heights. Although present in almost 10%, fractures and brain injury didn’t have long term sequelae. It is important to reinforce the importance of preventive and supervision measures.

400

Critical / Emergency Care

Knowledge Translation in Western Australia Tertiary Paediatric Emergency Department: An Audit Cycle of Effectiveness of Standard Education on Updated Bronchiolitis Guideline

Yee Lymn Yeo ¹ , Meredith Borland ¹ , Sharon O'Brien ¹ , Natasha Bear ²

¹ Emergency Department, Perth Children's Hospital (formerly Princess Margaret Hospital), Australia ² Telethon Kids Institute, Perth Children's Hospital, Australia

Background: Bronchiolitis is the most common cause of presentation and hospitalization for infants in Australia and New Zealand. Multiple clinical practice guidelines have been published to minimize unnecessary interventions in infants with bronchiolitis; however, the challenge is translating the knowledge and evidence into clinical practice.

Objective: The objective of this audit cycle was to determine if standard education process of the updated bronchiolitis guideline in a tertiary paediatric emergency department (ED) will reduce unnecessary interventions in infants with bronchiolitis.

Methods: A retrospective chart review was conducted in a tertiary paediatric ED of bronchiolitis presentations in 2015 (pre-education cohort of 465 presentations) and in 2017 (post education cohort of 343 presentations) after implementation of the updated guideline prior to the 2017 bronchiolitis season. The primary outcome was to evaluate the rate of interventions shown to be ineffectual in the updated guideline with secondary outcomes evaluating the rate of hospital, ICU admissions and the rate of multiple interventions versus single intervention.

Results: There was no difference found between 2015 and 2017 for chest x-ray, salbutamol, adrenaline, antibioitics and nasopharyngeal aspirates. There was increased use of glucocorticoids with increased odds of 60% (p=0.002) which was statistically significant. There was no difference with the number of interventions between 2015 and 2017. Hospital admissions were reduced from 2015 to 2017 (65.2% versus 56.0% respectively), with reduced odds of admission of 18% (p=0.008) that was statistically significant. There was no difference in ICU admissions.

Conclusion: The updated guideline through standard education process in ED did not show any statistically significant reduction of unnecessary interventions. Community initiated glucocorticoid administration, however, was increased. A larger multi-centered study will be beneficial to determine if the awareness of the guideline to the broader community would reduce unnecessary interventions as well as the challenge of translating current evidence into clinical practice.

1023

Endocrine & Diabetes

Management of Diabetic Ketoacidosis in Pediatric Intensive Care Unit

Habib Besbes ¹ , Imen Rhouma ¹ , Radhia Hadj Salem ¹ , Chebil Ben Meriem ¹ , Leila Ghedira ¹ , Chokri Chouchane ¹ , Slaheddine Chouchane ¹

¹ Pediatric Department, LR12SP17, Fattouma Bourguiba Hospital Monastir University, Tunisia

Background: In recent years, the incidence of type 1 diabetes has been increasing in children. The prognosis is related to acute metabolic complications, mainly ketoacidosis.

Methods: A retrospective descriptive study of patients admitted with inaugural ketoacidosis of type 1 diabetes to ICU of Pediatric Department in Fattouma Bourguiba Hospital of Monastir over 10-year period from 2008 to 2017. Results: A hospital incidence of the inaugural ketoacidosis was 2 per 1,000. The rate of the inaugural ketoacidosis among new diabetes cases was 22.52%. Fifty patients were collected: 23 girls and 27 boys. The mean age at admission was 6.6 ± 4.1 years. The mean time between the onset of symptoms and hospitalization was 15.51 ± 9.27 days. Polyuro-polydipsic syndrome and massive ketonuriawas found respectively in 98% and 82% of cases. Mean blood glucose at the time of diagnosis was 4.92 +/- 0.14 g/L. 16 children were treated according to modified Lestradet protocol and 34 childrenwere treated according to modified ISPAD protocol. Correction of blood glucose, ketonuria, pH and bicarbonates up to H8 were without significant difference between the two protocols (p0.05). The most common complication in the acute phase was hypokalemia (8 cases). No cases of cerebral edema were observed.

Conclusion: The inaugural ketoacidosis in children remains common in our region. Early diagnosis of type 1 diabetes is important to prevent complications.

632

Endocrine & Diabetes

A Rare Cause of Familial Pubertal Delay

Filipa Briosa ^1,2 , Rita Valsassina ^1,2 , Joana Soares ^2,3 , Marta Amorim ⁴ , Catarina Limbert ²

¹ Department of Paediatrics, Hospital Beatriz Ângelo, Portugal

² Department of Paediatric Endocrinology, Hospital Dona Estefânia, Portugal

³ Department of Paediatrics, Hospital de Santo André, Centro Hospitalar de Leiria, Portugal

⁴ Department of Genetics, Hospital Dona Estefânia, Portugal

Background: Delayed puberty (DP) is described as central or peripheral whether the problem lies in the hypothalamo–pituitary axis or in the gonads respectively. Idiopathic hypogonadotropic hypogonadism (IHH) is a central cause of DP due to a failure of gonadotrofin releasing hormone (GnRH) secretion impairing the physiological initiation of puberty.

Objective and Methods: We present three intra-familial cases of normosmic IHH (nIHH) related to a new association of two heterozygotic TARC3 mutations.

Case Report: The index case reports to a 16 years-old male with no pubertal development at this age. Later, two of his three sisters also presented complete hypogonadism. All cases had a normal sense of smell. Investigation showed low sex steroid and gonadotropin levels and no evidence of a mass lesion in cerebral MRI. The diagnosis of nIHH was supported by the identification of two heterozygous mutations on TACR3 gene in all subjects.

Conclusion: Congenital IHH is a very rare genetic disorder that if undiagnosed or untreated may lead to infertility associated to complete or partial absence of GnRH.TACR3 mutations have a critical importance on sexual maturation and are an important genetic cause of nIHH. The treatment of nIHH is based on sex steroids replacement, therefore promoting the pubertal development.

273

Endocrine & Diabetes

Exercise in Pediatric Type 1 Diabetes Mellitus: Which, Why and How.

Sofia Carneiro ¹ , Elisa Galo ²

¹ Área da Mulher, Criança e Adolescente, Centro Hospitalar Universitário de Lisboa Central - Hospital Dona Estefânia, Portugal

² Serviço de Pediatria, Centro Hospitalar do Oeste - Torres Vedras, Portugal

Background: Regular exercise has important health and social benefits for children and adolescents with type 1 diabetes mellitus (T1DM). Different exercise types, intensity and duration have significantly different effects on blood glucose (BG) control, and on the endocrine response to exercise. There have been significant recent Improvements in the knowledge of what underlies these changes and in the appropriate management to support the maintenance of euglycemia. This effects frequently presents additional barriers to exercise – fear of hypoglycemia and loss of glycemic control – increasing sedentary lifestyle in this population. Thus, exercise presents several important challenges to diabetes management, and requires special management by patients and clinicians, based on the knowledge of the impact of exercise on BG homeostasis.

Objective: To systematize the principles of exercise prescription in pediatric population with DMT1, including practical advices in the management of the metabolic control.

Methods: Systematic literature searching in PubMed database. Two types of literature were reviewed: guidance and published studies.

Conclusion: The most recent studies show the positive effects of exercise on cardiovascular risk management, helping to achieve target lipid profile, body composition and fitness and glycaemic goals. This population should be encouraged to practice a wide variety of exercise, with a careful management to ensure a good metabolic control. In general, aerobic exercise tends to lower BG concentrations, while anaerobic exercise tends to increase them. Both exercise types can be performed by children and adolescent with T1DM, and exercise prescription must be individualized, based on a sound understanding of the underlying physiology and adapted to the patient`s characteristics. The collaboration between the child/adolescent, healthcare provider, parents, school and the physical education instructor is crucial to a good adherence, promoting an optimal glycemic control during and after exercise.

640

Endocrine & Diabetes

Variations in How Youth with Type 1 Diabetes Transition to Adult Care

Roger Chafe ¹

¹ Pediatrics, Memorial University of Newfoundland and Labrador, Canada

Background: Young adults with type 1 diabetes are at increased risk for acute complications during their transition from pediatric into adult care. While there is likely considerable variation in how transition care is structured, there is little evidence about the impact that this variation may have on patient outcomes in early adulthood.

Objectives: To determine how young adults with type 1 diabetes transition into adult care in two Canadian provinces: Newfoundland and Labrador (NL) and Ontario (ON); to examine the impact of different models of transition care on outcomes for patients with type 1 diabetes; and to explore ways to better support patients with type 1 diabetes as they transition into adult care.

Design: Mixed methods: Administrative data and qualitative research.

Results: There are significant variations in how the transition to adult care was structured both within and between provinces. The rate of diabetic ketoacidosis (DKA) in ON youth (n=2,525) was higher in those aged 19-21 years compared to 15-17 years (12.0 vs. 9.3/100 person-years, p0.0018). There was no difference in the rate across transition-age in youth from NL (n=93). In NL, there was no dedicated transition program for much of the province. We helped develop and evaluate a single session transfer clinic. We found that such a clinic is a promising step towards improving patient and parent satisfaction without requiring new staff or significant outlays of new resources. It is unclear whether such a clinic will ultimately impact rates of hospitalizations or DKA.

Conclusion: In two Canadian provinces with different transition models and levels of resources, we found consistency in poor outcomes. Adverse events for this vulnerable population are high in both provinces and efforts to optimize system-level transition care are needed.

340

Endocrine & Diabetes

Precocious Puberty Caused by Ovarian Dysgerminoma with Gonadoblastoma in a 46,XX Girl: A Case Report

Iveta Dzivite ^1,2 , Margi Patel ^1,2 , Lasma Lidaka ²

¹ Endocrinology, Obstetrics and Gynaecology, Riga Stradiņš University, Latvia ² Endocrinology, Obstetrics and Gynaecology, Children's Clinical University Hospital, Latvia

Introduction: Gonadoblastoma is a rare tumour consisting of germ cells resembling granulosa and Sertoli cells. Sometimes these tumours contain stromal elements indistinguishable from lutein and Leydig cells. Almost all patients have 46,XY karyotype or various forms of mosaicism. Dysgerminomas are malignant, undifferentiated germ cell tumours that constitute 1% of primary ovarian neoplasms. Approximately 5% of dysgerminomas arise in abnormal gonads(from a gonadoblastoma).

Methods: The clinical case was analysed using patient history and clinical examination, laboratory, genetic, radiological and histological investigations, treatment methods and follow-up procedures.

Results: 6 years 4 months old female presented with early pubertal development, tall stature and a large abdominal mass. Height 135cm(+3.2 SDS) weight 30.45kg(+2.0 SDS). Tanner puberty stage III: Px4, Ax1, Ma3, Me1. Signs of virilization were present.

Laboratory data: significantly elevated estradiol, androstenedione, testosterone, alfa-fetoprotein, beta-hCG and AMH. LH and FSH – prepubertal level.

Bone age(RUS score-598 points) corresponded to an age of 12.2. MRI showed large heterogeneous mass(CC 25.1cm, LL 18.9cm, AP 10.3cm) on left ovary. Left periaortic lymph nodes(LN) were increased.

An operation was done after ovarian tumour was confirmed by MRI. Tumour and two additional LN were completely resected, biopsies were taken. Histological report: left ovarian dysgerminoma with metastasis in periovarian LN. However, the tumour was hormonally active, therefore, Sertoli cell component could not be excluded. Consultation from Finnish pathologists suggested dysgerminoma in combination with gonadoblastoma. Karyotype analysis of blood was 46,XX. Skin biopsy excluded mosaicism.

Four courses of chemotherapy were done. Further follow-up was provided by a multidisciplinary team. Blood analysis was taken to monitor hormone levels in each hospital visit. Skeletal scintigraphy, chest CT, abdomen MRI was done after chemotherapy – no metastasis was found.

Conclusions: A delayed precocious puberty diagnosis has caused this child significant health issues: hormonal disbalance, prognostic small adult height, therapeutical side effects from chemotherapy, increased risk for metastasis and secondary tumour development. For an early diagnosis of possible malignity, every child needs to be evaluated at least once a year (development, secondary sex traits by Tanner) and parents need to be educated. Successful diagnostic, therapeutic result and follow-up monitoring can only be achieved by a multidisciplinary team.

551

Endocrine & Diabetes

An Unusual Case of Delayed Puberty

Ana Esteireiro ¹ , Ana Ventura ¹ , Raquel Machado ¹ , Juliette Dupont ² , Brígida Robalo ¹ , Carla Pereira ¹ , Maria de Lurdes Sampaio ¹

¹ Unidade de Endocrinologia Pediátrica, Departamento de Pediatria, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Portugal ² Serviço de Genética, Departamento de Pediatria, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Portugal

Background: Kallmann syndrome (KS) is a rare congenital form of hypogonadotropic hypogonadism with anosmia/hyposmia.

Description: Female, 14 years old, without relevant personal or family history, referred because of pubertal delay (pubarche at age 13, without thelarche). She denied symptoms of neurological or other organs impairment, practice of strenuous physical exercise, restrictive eating behavior, illicit drug use and history of traumatic brain injury. The investigation performed before showed low estradiol levels and ultrasonography with small uterus, atrophic ovaries and horseshoe kidneys. She had good general condition, no dysmorphism, normal external genitalia and Tanner pubertal stage I (P3, B1, A3). Her growth velocity suffered progressive and slight slowdown after 10 years, but her height was still on the 50^th percentile. Complementary study showed low values of gonadotropins, 46XX karyotype and delayed bone age. LH-RH test revealed low values of FSH, LH and estradiol. KS was hypothesized and after a detailed inquiry anosmia/hyposmia was confirmed. Brain MRI showed bulbs and olfactory tracts atrophy and a small pituitary gland. Molecular analysis of the GnRHR and KAL1 gene by cyclic sequencing was normal, but the study proceeded to a Next Generation Sequencing (NGS) multigene panel approach that identified a heterozygous variant of uncertain significance in the KS gene FGF8 gene (c.283CG, p.Gln95Glu). Pubertal induction was performed with transdermal estrogens, and after menarche combined hormonal therapy was started.

Conclusions: In pubertal delay with hypogonadotropic hypogonadism and despite its rarity KS should be considered and anosmia/hyposmia should be searched for. Brain MRI shows typical anomalies in most cases. The genetic cause is recognized in only 30% of cases. Early diagnosis and hormone replacement prevent the physical and psychological consequences of pubertal delay. NGS approaches will contribute to reveal the molecular basis of a larger number of cases and may bring better understanding and classification of these disorders.

659

Endocrine & Diabetes

A Twenty Month-Old Boy With Russell-Silver Syndrome (RS Sy)

Aleksandra Janchevska ¹ , Velibor Tasic ² , Zoran Gucev ¹ , Olivera Jordanova ³ , Thomas Eggermann ⁴

¹ Endocrinology, University Children's hospital, Macedonia

² Nephrology, University Children's hospital, Macedonia

³ Biochemical laboratory, University Children's hospital, Macedonia

⁴ Institute für Humangenetik, Uniklinik RWTH, Germany

Background: The alterations in a telomeric imprinting region 1 (ICR 1) in 11p15 chromosome and a maternal uniparental disomy of chromosome 7 (upd(7)mat) may not be detected by molecular analyses in approximately 50% patients with characteristic features of Russell-Silver syndrome.

Patient and Methods: A twenty month-old boy with triangular shaped face, midface hypoplasia, prominent forehead, wide, but thin mouth, small chin, low positioned ears, occipitofacial circumference (OFC) (-2.3 SDS) had low weight (-3.75 SDS), short stature (-4.6 SDS) and body asymmetry with bigger left side and longer left limbs for 1cm. The boy was born with intrauterine growth retardation (IUGR), birth weight (-4.8 SDS) and birth length (-1.7 SDS), in 38th gestational week (GW). The diagnostic assessment was achieved by clinical features, ultrasound survey, hormonal and molecular analyses.

Results: The IGF1, IGF BP3 and thyroid hormones were within reference range. The tests for pituitary reserve revealed a peak of 8.64ng/ml. The ultrasound survey revealed hypoplastic right kidney and mild hypertrophy of the myocard. Karyotype was 46, XY. Multiplex ligation probe amplification (MLPA) and a methylation analysis (MS-MLPA) did not detect hypomethilation of the ICR1 or a upd(7)mat. The growth hormone treatment is planned to be commenced.

Conclusions: Herein we present a twenty month-old IUGR born boy with typical clinical features of Russell-Silver syndrome. The molecular analyses unfortunately did not confirm hypometilation in the critical region of chromosome 11p15 or maternal uniparental disomy of chromosome 7.

925

Endocrine & Diabetes

Use of FGMS (Freestyle Libre) in Children and Young People with Type 1 Diabetes Mellitus-a UK DGH Experience

Surendran Chandrasekaran ¹ , Sanjana Kattera ¹ , Nosheen Aman ¹

¹ Paediatrics, East Cheshire NHS Trust, UK

Data was retrospectively collected from Electronic Patient Records for a 12-month period between June 2017 and June 2018. There were 100 CYP’s with T1DM, of which 50 were commenced on FGMS. Funding sought appropriately before being commenced on sensor. CYP’s and families were also made aware of the fact that funding can be withdrawn after a 6-month trial period if outcomes were unmet. HbA1c at the start of the trial was compared with HbA1c at 3, 6 and 9 months.

Results: The HbA1c at the start of the trial was 65.1 mmol/mol; which showed an 8.1% improvement at 3 months, 12.3% at 6 months and 12.8% at 9 months. 67% discontinued the use of the FGMS, as qualitative evidence reported it to be unreliable. 2 of these children, recommenced the FGMS.

			Total (n=50)
Average age at start of trial	13.6	11.3	12.8
HbA1C pre-sensor (mmol/mol)	75.1	64.2	65.1
HbA1C at 3 months (mmol/mol)	88.5	62.4	59.8
HbA1C at 6 months (mmol/mol)	58.3	58.3	57.1
HbA1C at 9 months (mmol/mol)	52.2	57.7	56.8
June 2018-19, 6 new patients were commenced FGMS
HbA1C pre-sensor (mmol/mol)	84.5	65	71.5
HbA1C at 3 months (mmol/mol)	40	54.3	49.5
HbA1C at 6 months (mmol/mol)	46	52.3	50.5

Conclusion: Our data shows a reduction in HbA1C after 3 months of continuous sensor use which is maintained at 6 and 9 months. The improvement in HbA1c is marked if the sensor was started within 6 months of diagnosis compared to those who have had T1DM for longer. Education is crucial before the start of sensor so that families and CYP’s are well equipped to get the best out of it. The use of outcomes-based criteria as set by the CCG’s acts to incentivise families, CYP’s and HCP’s to improve control.

228

Endocrine & Diabetes

Precocious Puberty: When the Cause is not Idiopathic

Ana Luísa Leite ¹ , Elisa Galo ³ , Ana Antunes ⁶ , Brígida Robalo ⁴ , Filipa Espada ⁷ , Sofia Castro ² , Catarina Limbert ⁵

¹ Unidade de Endocrinologia e Diabetologia Pediátrica, Centro Hospitalar de Vila Nova de Gaia / Espinho, Portugal

² Serviço de Pediatria, Centro Hospitalar Barreiro Montijo, Portugal

³ Serviço de Pediatria, Hospital da Luz, Portugal

⁴ Unidade de Endocrinologia Pediátrica, Centro Hospitalar Universitário Lisboa Norte, Portugal

⁵ Unidade de Endocrinologia Pediátrica, Centro Hospitalar Universitário de Lisboa Central, Portugal

⁶ Unidade de Endocrinologia Pediátrica, Hospital de Braga, Portugal

⁷ Unidade de Endocrinologia Pediátrica, Hospital Pedro Hispano, Portugal

Precocious Puberty is defined by the onset of pubertal development at an age 2-2,5 standard deviations earlier than the normal population, which means before nine years old in boys and eight years in girls. Central Precocious Puberty (CPP) is diagnosed when the hypothalamic-pituitary axis is activated. Although the majority of CPP causes are idiopathic, secondary causes should be discharged because of pathologic conditions with significant risk of morbidity and even death.

Aims: To evaluate the prevalence of secondary CPP in the Portuguese population in the last five years, to characterize anthropometric and imagological features and identify its clinical predictors.

Methods: Cross-sectional study from a National Digital Database, enrolled from eleven Public and Private Pediatric Endocrine Departments. Cases reported from January 2013 to December 2017. Statistical analysis was performed using SPSS^TM 23.0 version.

Results: A total of 210 cases presented CPP during the last five years. 187 (89%) were female. The median age of diagnosis was 6,5 years old in idiopathic CPP and 4,8 years old in secondary CPP (p=0,002). Only 146 (69.5%) enrolled hypophyses/cranial image and secondary cases of CPP were identified in 38 patients (26%). Tumors were diagnosed in ten cases (26,3% of the secondary CPP) and the most frequent was hamartoma in 60%. From the secondary cases of CPP only 17 were male (12%) and there were no significant differences among gender when considering idiopathic/secondary cases. There was a negative correlation between the age of clinical manifestations (p0.001) and secondary CPP.

Conclusions: CPP is more frequent in girls, either in idiopathic or secondary cases. The yearly age of clinical manifestations is a better predictor than gender in the diagnosis of secondary CPP.

814

Endocrine & Diabetes

Thyroid Status and Serotonin Production in Children with Impaired Growth and Neuropsychological Development

Hanna Shlieienkova ¹ , Marina Mamenko ²

¹ Paediatrics № 2, V. N. Karazin Kharkiv National University, Ukraine

² Paediatrics № 2, Shupyk National Medical Academy of Postgraduate Education, Ukraine

Thyroid hormones (TH) are essential for the development and maturation of the central nervous system. Serotonin, 5-hydroxytryptamine (5-HT), is an important chemical and neurotransmitter in the human body. It appears to play a key role in neural activities and social behaviors. The effects of TH and serotonin during development of CNS appear similar. Transient hypothyroidism is common in children with impaired CNS maturation. The levels of serotonin production in these children remain unclear.

Objective: To evaluate serotonin levels and thyroid status in children with impaired growth and neuropsychological development.

Materials and Methods: Target group: 118 children 0–3 years old from orphanage.

Methods: physical, psychomotor development assessment; thyroid status by TSH, TT4, TT3 serum-based measuring (IMA); peripheral blood serotonin measurement («IBL Hamburg», Germany).

Results: All children were accepted in to an orphanage at the age 2.5-3.5 months. They had impaired physical development (44.9%), delayed psychomotor development (95.7%), decreased coefficient of neuropsychological development (82 points). TSH level median was 3.21mU/l [QR: 3.1; 3.8]. Negative linear correlation by PPMCC between TSH levels and coefficients of neuropsychological development was detected (ρ=−0.436, p=0.001). In children with minimal thyroid dysfunction median serotonin level was 204.1nmol/ml [QR: 202.1; 206.1], vs in children with optimal thyroid function 185.1 nmol/ml [QR: 177.7; 189.7], р

Conclusions: In young children, disorders of physical and neuropsychological development are associated with transient hypothyroxinemia and hyperthyrotropinemia. Unlike adults, hyperthyrotropinemia is associated with increased serotonin production. Perhaps such increase in serotonin production is an additional mechanism aimed at completing the maturation of the nervous system.

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Endocrine & Diabetes

Blurry Vision in Type 1 Diabetes

Maria Ines Marques ¹ , Antonio Guerra ¹ , Bruno Pina ² , Sara Carrasquinho ² , Isabel Fernandes ¹

¹ Pediatrics, Hospital Santo Espirito Santo, Portugal ² Ophthalmology, Hospital Santo Espirito Santo, Portugal

The development of cataracts at the onset of type-1 diabetes mellitus (T1DM) is an uncommon complication in the pediatric population (prevalence of around 1%). It most often occurs as one of the first manifestations or within 6 months of T1DM diagnosis.

Our aim is to describe two cases of adolescents with a recent diagnosis of type-1 diabetes mellitus that were diagnosed with a complication of posterior subcapsular cataracts.

Case 1

A case of an 18-year-old girl, with complaints of reduced visual acuity in both eyes, about one year after T1DM diagnosis, under intensive insulin therapy and excellent glycaemic control (HbA_1C6.1%). After ophthalmological examination, she was found to have bilateral posterior subcapsular cataracts (PSC), that were not present at the time of a routine diabetic eye screening at diagnosis.

The patient underwent cataract surgery and intraocular lens implantation with improved visual acuity.

Case 2

A case of a 17-year-old girl who complained of blurry vision six months after T1DM diagnosis, during an ophthalmological screening. Good metabolic control of diabetes was observed during this time (HbA_1C6.7%).

The patient had a unilateral PSC of the left eye. About one year after the first diagnosis she developed PSC of the right eye. She underwent cataract surgery and intraocular lens implantation with improved visual acuity.

Conclusion: Type-1 diabetic cataracts have been found to have a female preponderance, while the pathogenesis remains unclear, it seems to be related to poor metabolic control or long history of symptoms prior to diagnosis. In these cases, good metabolic control did not appear to prevent cataract formation.

Visual impairment occurring early in T1DM in pediatric age should raise a suspicion of cataracts and ophthalmologic evaluation should be prompt.

Close monitoring of the fundi for retinopathy following cataract surgery is essential, as proliferative retinopathy may develop rapidly after the surgery.

537

Endocrine & Diabetes

Recurrent Vomiting in Young Infant: A Challenge

Tania Mendo ¹ , Catarina Borges ¹ , Gabriela Reis ¹ , Maria Carlos ¹ , Fatima Furtado ¹ , Sónia Fernandes ¹ , Graça Seves ¹ , Aniceta Cavaco ¹

¹ Pediatria Medica, Hospital José Joaquim Fernandes, Portugal

Background: Recurrent vomiting in infants may have several etiologies. Among the most common medical causes are gastroenteritis, urinary tract infections or CNS infections. Besides that, surgical causes such as hypertrophic pyloric stenosis or intestinal obstruction should also be considered. Therefore, diagnosis is not always easy and may require extensive research.

Case Report: Two-months-old male, with previous history of reccurent vomiting, observed in the emergency department with vomiting, poor weight gain, and asymptomatic hyponatremia. Family history of hypothyroidism and rheumatoid arthritis. At physical examination, he was hydrated and hemodynamically stable.

Blood tests showed Hb 11.7 g/dL, Ht 34.6%, WBC 20510 U/L, N 28.6%, L 59.1%, Na + 120 mmol / L, K + 6.94 mmol / L and the abdominal ultrasound was normal.

He was admitted for IV hydration and correction of hyponatremia.

From the additional investigation performed, the most common causes of vomiting were excluded, as well as tubulopathies, SIADH and hypothyroidism. The diagnosis of congenital hyperplasia of the adrenal gland was considered the most likely, and lately confirmed.

He started therapy with hydrocortisone, and then transferred to a tertiary hospital (Endocrinology Unit) for further care.

Conclusion: With this case the authors aim to draw the attention to a less common cause of recurrent vomiting in young infants. Congenital adrenal hyperplasia is an inherited autosomal recessive disease, caused by the deficiency of enzymes involved in cortisol synthesis. About 95% of cases are caused by 21-OH deficit. In the neonatal period, the diagnosis can be suspected in the presence of sexual ambiguity or acute salt-losing crisis. Treatment is essential to ensure appropriate growth, puberty and absence of long-term complications.

503

Endocrine & Diabetes

Neurological Symptoms at Presentation: What it could be?

Sofia Brandão de Miranda ¹ , Joana Vilaça ¹ , Sofia Martins ¹ , Marina Pinheiro ¹ , Vera Baptista ¹ , Maria João Magalhães ¹ , Ângela Oliveira ¹

¹ Pediatria, Hospital de Braga, Portugal

Background: Neurological symptoms can have multiple causes, such as structural changes of the central or peripheral nervous system as well as metabolic, hydroelectrolytic or even psychiatric disorders.

Objectives / Methods / Results: A 12-years-old adolescent presented at emergency service (ES) with acute onset of blurred vision, drowsiness, headache and speech difficulties. He was tachycardic but normotensive, emaciated, with slurred speech and dry mouth and objective weight loss of 22% in a week.

Blood test revealed hyperglycemia (glucose 1763mg/dL), moderate metabolic acidosis (pH 7.169, HCO3 - 12.1mmol/L), high blood ketone levels (ketone bodies 7mmol/L), acute renal failure (creatinine 2.3mg/dL, urea 108mg/dL) and sodium level 135mmol/L (corrected sodium 161). Head computed tomography without cerebral edema or structural changes.

Based on results it was made the diagnosis of Type 1 Diabetes mellitus (T1DM). Retrospectively, patient`s mother reported polyuria, polydipsia and polyphagia with several weeks of evolution, and recent pollakiuria that motivated a previous visit to the ES. Fluid therapy and intravenous perfusion of insulin was started. Careful and frequent adjustments were needed, nevertheless he developed important hypernatremia (corrected sodium 175 mmol/L). Progressively, he presented improvement in glycemia, ketoacidosis, renal function and dehydration, with disappearance of neurological symptoms.

Subsequent study with decreased C-peptide and insulin, increased GAD65 and anti-insulin antibody, and normal levels of anti-zinc transporter protein 8 autoantibodies, thyroid function and lipid profile.

At discharge he had no analytic changes. Weight gain of 21%, with no other changes at physical examination.

Conclusion: The elevated glycemia and the consequent hydroelectrolytic disorders following the treatment constituted a challenge. It was important to control the rapid rise in hypernatremia to avoid serious consequences as brain shrinkage, intracerebral hemorrhage and demyelination. This case also alert to the importance to a earlier diagnosis of T1DM and to some less frequent and even confounder symptoms in an inaugural episode.

794

Endocrine & Diabetes

Evaluation of Nipple Discharge in Adolescence – A Case Report

Rita Gomes ¹ , Alicia Rebelo ² , Ivete Afonso ³ , Patrícia Santos ³ , Marcelo Fonseca ³ , Filipa Espada ³ , Ana Moreira

¹ Pediatrics, Centro Materno Infantil do Norte, Centro Hospitalar Universitário do Porto, Unidade de Saúde Local Matosinhos, Portugal

² Pediatrics, Hospital da Senhora da Oliveira Guimarães, Unidade de Saúde Local Matosinhos, Portugal

³ Pediatrics, Unidade de Saúde Local Matosinhos, Portugal

Background: Nipple discharge is uncommon in children and adolescents. Milky discharge is characteristic of galactorrhea which is typically bilateral and may be secondary to pregnancy, medications, endocrine tumors (pituitary adenoma), endocrine abnormalities (hypothyroidism, hypogonadism), mechanical stimulation or psychological factors.

Case Report: A 17-year-old girl was referred to a Pediatric Endocrinology appointment at ULSM for nipple discharge in the last six months. She had recent complaints of emotional lability and anxiety and a family history of depression. No menstrual irregularities, hot flashes, vaginal dryness, headaches, visual disturbances or other symptoms were mentioned. Pregnancy and medication-related causes were excluded. On physical examination, a bilateral milky nipple discharge was elicited by manipulation. No skin changes, asymmetries or masses were evident on breast examination. No areas of tenderness or pain nor axillary or supraclavicular lymph nodes were palpable. Laboratory tests showed mild elevation of serum prolactin levels, normal LH, FSH, T4 and TSH. Magnetic resonance imaging (MRI) showed a 5 mm diameter microadenoma of the anterior pituitary and an asymmetry of the sellar diaphragm with a left shift of the pituitary stalk. After discussion with neurosurgery specialists, she was started on cabergoline 0,25mg twice a week. After 3 months, the galactorrhea was eliminated, there was normalization of the prolactin concentration and there was no significant change in size of the sellar mass.

Conclusions: It has been reported in the literature that depressive symptoms may be associated with mild elevations of serum prolactin levels. Notwithstanding, an MRI of the pituitary should be performed in symptomatic patients with any degree of hyperprolactinemia. Prolactin concentrations 200 ng/mL generally identify a lactotroph adenoma; values that are between 20 and 200 ng/mL could also be due to any other sellar mass. Children and adolescents with spontaneous nipple discharge without an obvious cause should be referred to an appropriate specialist.

1003

Endocrine & Diabetes

Addison’s Disease in a Girl with Family History

Mafalda Moreira ¹ , Diana Alba ¹ , Ana Garrido ¹ , Mariana Martinho ² , Ana Catarina Chaves ² , Carla Brandão ¹

¹ Pediatric Department, Centro Hospitalar Tâmega e Sousa, Portugal

² Endocrinology Department, Centro Hospitalar Tâmega e Sousa, Portugal

Background: Primary adrenal insufficiency is a rare condition especially in pediatrics’ population and can be fatal if not identified and treated on time. The usual presentation includes constitutional and gastrointestinal symptoms, cutaneous hyperpigmentation and hyponatremia. The treatment consists in hormonal replacement throughout life.

Clinical case: We present the case of a 16 years old female, admitted at the emergency department with asthenia, weight loss and daily vomiting with a month evolution. In admission, she presented cutaneous hyperpigmentation, without dehydration signs. Laboratory results showed hyponatremia with hyperkalemia and hypochloremia, decreased cortisol levels with increased levels of corticotropin and increased levels of renin with decreased aldosterone levels. She started treatment with hydrocortisone and fludrocortisone with favorable response. Additional investigation evidenced a positive family history for autoimmune primary adrenal insufficiency in a sibling.

Discussion: This case is illustrative of an uncommon disease, whose diagnosis requires a high degree of suspicion. The presence of cutaneous hyperpigmentation in a patient with constitutional symptoms and vomiting was the key to the diagnosis, as well as the presence of hyponatremia with hyperkalemia. The family history, although uncommon in this disease, was also a clue to the diagnosis.

803

Endocrine & Diabetes

Re-interpreting the Appropriate Growth Hormone Response to Hypoglycaemia

Uzair Athar Khan ¹ , Ying Wong ¹ , Shahir Ul Azam ¹ , Ophelia Blake ² , Anne Marie Murphy ^1,2 , Orla Neylon ^1,3 , Clodagh S O'Gorman ^1,3

¹ Paediatric department, University Hospital Limerick, Ireland ² Department of biochemistry, University Hospital Limerick, Ireland ³ Paediatric department, Graduate Entry Medical School University of Limerick, Ireland

Background: The “Newcastle work-up” is laboratory investigation to elucidate metabolic or endocrine causes of hypoglycaemia. One of the tests it measures is growth hormone GH,which should increase during hypoglycaemia. In our recent clinical experience, GH levels have been sub-optimal during hypoglycaemia. The cut off value for growth hormone levels is 7mcg/L,reading below is deemed suboptimal and warrant clinician to investigate further.

Aims/Objectives: To identify if the pre-set cut-off value for GH during hypoglycaemia during Newcastle work-up is appropriate and likely or not to identify GH deficiency. The study population was all paediatric patients attending the Paediatrics Department UHL with GH levels over 12 months from January to December 2018, inclusive.

Methods: Retrospective review from a prospectively collected database of glucose and GH,including growth velocity, centiles, mid-parental height ranges and IGF-1 during hypoglycaemia in the Newcastle work-up. GH levels were subdivided into five subgroups: 1; 1-2.9; 3-4.9; 5-6.9; 7 mcg/L.

Results: Forty Newcastle work-ups were performed on N=40 patients during the study period;GH results according to 5 subgroups are shown in Figure 1. N=3/39 (8%) GH levels were 7 mcg/L, which is the pre-defined normal range result. The mean GH was 3.83 and median GH 3.19 mcg/L.IGF-1 level was available on 12/40; 11/40 were normal. N=1/40 IGF-1 was low and this patient had decelerating height centile, GHST was subsequently normal.N=19/40 patients had normal growth velocity and growth centiles,Data on remaining patients are incomplete or pending.GHST was performed on 3/40 children.All had normal GHST results.

Conclusion: These data are limited and some results are pending. But no patient has documented GH deficiency or loss of height centiles, despite only N=3/40 having adequate GH during hypoglycaemia during Newcastle testing.

Recommendations: These data suggest that the optimal level for GH during testing for causes of hypoglycaemia should be re-evaluated in further and larger prospective studies

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Endocrine & Diabetes

Hirsutism and Oligomenorrhea, Not Always Polycistic Ovary Syndrome

Silvia Gallego Gutierrez ¹ , Eva Pedrero Segura ¹ , Ana Isabel Valdivielso Martinez

¹ Malaga-GuadalHorce District, Primary Care Attention, Spain

Background: Hirsutism is excess terminal hair that commonly appears in a male pattern in women. The most common cause of hirsutism is polycystic ovary syndrome. Other causes are idiopathic hyperandrogenemia, idiopathic hirsutism, adrenal hyperplasia, iatrogenic hirsutism, thyroid dysfunction, Cushing syndrome and androgen-secreting tumors. Congenital adrenal hyperplasia is a group of autosomal recessive disorders encompassing enzyme deficiencies in the adrenalsteroidogenesis pathway that lead to impaired cortisol biosynthesis.

Objective: We present a case of non-classic congenital adrenal hyperplasia (NCAH) in a 13 year old female who presented to us with hirsutism and oligomenorrhea.

Methods: Case reports.

Results: A 13-years-old girl was referred for evaluation for hirsutism. Height 168 cms, weight 54 kgs and BMI of 19.13. Distribution and extent of hirsuitism assessed by modified Ferriman-Gallway score was 11 (moderate hirsuitism). She has not rapid onset of symptoms, signs of virilization, and she had not palpable abdominal or pelvic mass. The patient had already consulted on other occasions with the diagnosis of idiopathic hirsutisms. Ultrasonography showed uterus and bilateral ovaries normal in size shape and ecopattern. There was no detectable ovarian or adrenal mass. Androstenedione, dehydroepiandrosterone sulfate and 17-hydroxyprogesteronewere elevated (8.82ng/ml, 3421 ng/ml and 38.1 ng/ml respctively). Estradiol (54 pg/ml), FSH (10.61 mIU/ml), T4 (16.56pmol/L) and TSH (4.49 mUI/ml) were normal. A genetic study was carried out, finding a mutation inthe 21-hydroxylase (CYP21A2).

Conclusion: Women presenting with signs and symptoms of androgen excess should be investigated about NCAH. The majority of NCAH can be associated with three distinct enzyme defects: defects of 21-hydroxylase, 11β-hydroxylase and 3β-hydroxysteroid dehydrogenase. The first one is the most common. This case emphasizes the importance of making the differential diagnosis of hirsutism with interdisciplinary follow-up in patients with hormonal diseases.

789

Endocrine & Diabetes

A Case Report of a Congenital Adrenal Hyperplasia in a Seven-day-old Boy

Miguel Paiva Pereira ¹ , Filipa Marujo ¹ , Cláudia Fernandes ¹ , Ana Cristina Monteiro ¹ , Cristina Matos ¹

¹ Centro da Criança e do Adolescente, Hospital CUF Descobertas, Portugal

Congenital Adrenal Hyperplasia (CAH) is an inherited autosomal recessive disorder characterized by a deficiency of enzymes involved in the synthesis of cortisol, mineralocorticoids or androgens. Its incidence is 1:18000 and the most common type is 21-hydroxylase deficiency. Classic CAH can result in a salt-losing form or a simple virilizing form.

A 7-day-old newborn was brought to the emergency department for prostration and decreased intake. Forty-six-year-old mother, with 2 previous miscarriages. Pregnancy without intercurrences. Elective caesarean section at 38 weeks, Apgar score 6/9/10 and a low birth weight (2335g). He was lethargic and pale, with a 9% weight loss. Urinalysis with 25 leukocytes, hyponatremia (120mmol/L), hyperkalaemia (9.4mmol/L) with electrocardiographic changes and hypoglycaemia (25mg/dL). He was admitted to Neonatal Intensive Care Unit level II, initiating intravenous antibiotics (ampicillin, gentamicin and cefotaxime), bicarbonate, hydrocortisone and ion-exchange resins. Uroculture was positive for Escherichia coli. Investigation revealed high levels of adrenocorticotropic hormone (566pg/mL), cortisol (28.8mcg/dL) and 17-OHP (241.9ng/mL), confirming the diagnosis of CAH. Fludrocortisone (0.3mg/day) was started. Progressive weaning of intravenous corticosteroid and passage to the oral route according to guidelines, with successive relapses and ionic imbalances. By the 34^th day, clinical and analytical stabilization was achieved under hydrocortisone 61,4mg/m2, NaCl 6,2meq/kg/h and fludrocortisone, and was discharged home. At present, he remains stable, without adrenal crisis and reducing hydrocortisone (22,7mg/m2). The genetic study is underway.

CAH is a serious condition at risk of impending decompensation. Clinical severity and stabilization are related to the initial enzymatic deficit, so corticosteroid doses and the weaning rhythm should be individualized and the patient monitored closely. CAH has non-specific clinical manifestations in newborns, so a high suspicion index is needed facing a presentation before the third week of life with no virilization signs, as in this case, whose severity and precocity were triggered by an infectious event.

610

Endocrine & Diabetes

Newborn Pseudohypoparathyroidism: A Case Report

Vicente Rey y Formoso ¹ , Sofia Fernandes ¹ , Rita Santos Silva ^2,3 , Carla Costa ^2,3 , Miguel Leão ⁴ , Cíntia Castro Correia ^2,3 , Manuel Fontoura ^2,3

¹ Pediatrics Department, Maternal and Pediatrics Center – São João University and Hospital Center, Portugal

² Pediatric Endocrinology Unit - Pediatrics Department, Maternal and Pediatrics Center – São João University and Hospital Center, Portugal

³ Pediatrics Department, Porto Medical School, Portugal

⁴ Genetics Department, São João University and Hospital Center, Portugal

Background: Pseudohypoparathyroidism(PHP) represents a group of disorders whose main feature consists in a resistance to Parathormone(PTH) action. The most common subtype of PHP is Ia, caused by a mutation in the GNAS1 gene. It is inherited as an autosomal dominant trait but, because of paternal gene imprinting, different phenotypes occur depending on the origin of the mutated gene. When paternally inherited, subjects only manifest the physical syndrome – Albright’s Hereditary Osteodystrophy (obesity, rounded face, brachydactyly, subcutaneous ossifications…). When maternally inherited, in addition to those physical characteristics, multiple hormone resistance (PTH, Thyroid-stimulating Hormone(TSH), gonadotropin…) occurs – PHP-Ia.

Objectives: Case report of a newborn with PHP-1a presenting with hypothyroidism – the importance of family history, a high suspicion level and thorough laboratory workups.

Methods: Reviews of the subject’s and his family’s clinical records.

Results: A 27-year-old female gravida (37 weeks gestation), with genetically proven AHO (paternally inherited GNAS1 mutation), gave birth to a 2360g male that presented with early signs of respiratory distress, for which he was admitted in the NICU. Respiratory symptoms resolved gradually. Because of the family history, at day 6, thyroid hormone testing was requested, showing a free-T4 level of 0.67mg/dL and a TSH concentration of 13.02 IU/mL. Hypothyroidism/TSH insensitivity was diagnosed – levothyroxine therapy was initiated, and thyroid function levels normalized in the following days – discharge on the 18th day of life. Genetic testing revealed the newborn carried the same mutation as the mother, confirming the diagnosis of PHP-1A. An ultra-sound showed a normal but relatively small thyroid gland. Phosphocalcium metabolism and thyroid function (under levothyroxine) remain normal at 3-month follow-up.

Conclusion: While PTH resistance is the main feature of PHP-Ia, TSH resistance may become apparent before hypocalcemia develops, and may be indistinguishable from primary hypothyroidism. Early treatment and continuous monitoring of these patients’ hypothyroidism is paramount in preventing future complications.

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Endocrine & Diabetes

Impact of Growth Hormone Supplementation on Adult Height in Turner Syndrome

Liliana Sá ¹ , Sofia Simões Ferreira ² , Maria Adriana Rangel ² , Ana Luísa Leite ² , Rosa Arménia Campos ²

¹ Pediatrics and Neonatology, Centro Hospitalar de Entre Douro e Vouga, Portugal

² Pediatrics and Neonatology, Centro Hospitalar Vila Nova Gaia / Espinho, Portugal

Background: Short stature is the most common phenotypic feature of Turner Syndrome (TS). Treatment with Growth Hormone (GH) is recommended to increase growth velocity (GV) and final adult height.

Objective: To describe patients with TS who underwent treatment with GH in a level II hospital in the north region of Portugal.

Methods: Retrospective observational study.

Results: Eight patients with TS underwent treatment with GH with a mean age at diagnosis of 4.5 ±3.9 years. Chromosome analysis demonstrated 3 cases of X-monosomy and 5 cases of mosaicism. None had cardiac pathology and 4 had hypothyroidism. Baseline mean height was 106.1 ±16.5 cm (SDS -2.0 ±0.69), with annualized GV of 4.7 ±1.7 cm/year, and adjusted mid-parental height (MPH) of 158.7 ±4,7 cm (SDS -0.56 ±0.78). Treatment with GH was started on average at 6.7 ±3.3 years of age (subcutaneous daily injection, mean dose of 48 mg/Kg/day). Currently, the eight patients, with a mean age of 15.0 ±4.3 years, present a mean height of 145.5 ±10.7 cm (SDS -1.85 ±0.65), with a gain of about 49 ±10.7 cm. Throughout the treatment the difference between the SDS of the MPH and the SDS of the stature decreased (1.52 ±0.70 to 1.29 ±1.22), and five patients reached the expected MPH-SDS. The growth was about 5.7 ±1.3 cm per year of treatment, with the greatest increase during the first year (mean of 9.0 ±1.8 cm). Five patients had already completed treatment at a mean age of 14.6 ±0.9 years. After an average of 9.4 years of treatment they reached a final height of 149.0 ±3.9 cm (SDS -1.85 ±0.66).

Conclusion: Treatment with GH should be instituted as soon as possible in order to optimize adult height.

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Gastroenterology & Nutrition

Maternal Orthorexia Nervosa Causing Severe Ketoacidosis and Methylmalonic Acidura in Her Offspring

Said Abozaid ¹ , Mary Abdo ¹ , Galit Tal ² , Mariam Daher ¹ , Noa Mandel-Shorer ³ , Elena Dumin ² , Wael Nasser ¹ , Hanna Mandel ^1,4

¹ Department of Pediatrics, Baruch Padeh Medical Center affiliated to the Faculty of medicine in the Galilee Bar-Ilan University, Israel

² Metabolic Unit, Ruth Rappaport children's hospital, Rambam Health Care Center, Israel

³ Hematology-oncology division, Ruth Rappaport children's hospital, Rambam Health Care Center, Israel

⁴ Department of Genetics, Western Galilee Medical Center, Israel

Introduction: Orthorexia Nervosa (ON) is an eating disorder describing patients with obsession for healthy food, in contrast to patients with anorexia and bulimia who worry about its quantity. ON may lead to a state of starvation due to strict diets and shortage of essential nutrients leading to various medical complications as seen in anorexia. To-date, only anecdotal cases on ON in adults were reported. We report for the first time a baby with episodes of apathy and severe ketoacidosis. History of the baby`s and his mother`s nutrition led us to suspect ON in the mother causing his critical condition.

Case report: A 17-months-old baby presented with few days history of lethargy and refusal to eat after an attempt of weaning breastfeeding. He had been mainly breastfed and rarely consumed solid food. A detailed dietary history revealed that the mother ate only vegan diet during nursing and provided her baby a strict "vegan, healthy uncooked diet omitting animal products and fortified babys` formulas". The baby was lethargic, pale, hyperpneic, had hypotoia with hyperrexflexia. His growth centiles were below the 3^rd percentile. He could not role over nor sit unassisted. Laboratory studies revealed pancytopenia and severe ketoacidosis. Clinical and biochemical features suggested an inborn error of metabolism (IEM). Urinary organic acids revealed severe ketoacidosis and methylmalonic aciduria. Slow refeeding program including multiple vitamins and vitamin-B12 resulted in resolution of all abnormalities.

Conclusion: We describe for the first time ON in a mother causing life-threatening disorder in her child. We designate this condition as "Maternal ON" similar to the notion of "Maternal phenylketonuria" (PKU) wherein high phenylalanine levels in a PKU mother who does not keep restricted diet, harms her offspring. Pediatricians and dieticians should be aware to Maternal ON mimicking IEM presenting as growth and development delay and/or severe metabolic abnormalities.

532

Gastroenterology & Nutrition

Acute Upper Gastrointestinal Bleeding Associated with Epstein-Barr Virus Infection

Carolina Amaro Goncalves ¹ , Ana Lança ² , Madalena Sasseti ³ , Marta Almeida ³ , Filipa Nunes ³ , Paulo Calhau ³

¹ Pediatric, Pediatric Department, Hospital de Santa Maria - Centro Hospitalar Universitário de Lisboa Norte, EPE, Lisbon, Portugal, Portugal

² Pediatric, Pediatric Department, Hospital de São Francisco Xavier - Centro Hospitalar de Lisboa Ocidental, EPE, Lisbon, Portugal, Portugal

³ Pediatric, Pediatric Department, Hospital Garcia de Orta EPE,Almada, Portugal, Portugal

Epstein–Barr virus (EBV) is a herpes virus responsible for a lifelong latent infection in almost every adult. Primary infection generally occurs subclinically in childhood, but if infection is delayed it frequently causes a self-limited clinical syndrome of infectious mononucleosis.Classic symptoms include pharyngitis, fever and lymphadenopathy. However, EBV infection may exhibit numerous rare, atypical and threatening manifestations involving different organ systems.Predominant gastrointestinal involvement is rarely documented.We present an unusual case of acute EBV infection–associated hemorrahagic gastropathy.

Case report: A previously healthy 12-year-old male child presented to the emergency department with a 4-day history of acute onset of malaise, weakness, anorexia and 2 episodes of lypotimia followed by 3 melena stools.There was no fever, weight loss, abdominal pain, vomiting or sore throat.He reported no NSAID, drugs or alcohol history. Physical examination revealed a pale face, afebrile with tachypnea and tachycardia.There was no skin rash, jaundice,lymphadenopathy and the abdomen was soft with no rebound tenderness or hepatosplenomegaly. A complete blood count showed a hemoglobin of 6.8g/dL, a normal platelet count, and white blood cell count of 4200/μL with 27% lymphocytes.The international normalized ratio, partial thromboplastin time and liver function test results were normal. He received transfusion of red blood cells and after being excluded Meckel´s diverticulum, an upper GI endoscopy revealed an increased friability of the mucosa and hemorrhagic gastropathy.Treatment with proton pump inhibitors were initiated and was maintained for 8 days with favorable results.An upper endoscopy before the discharge reveled a completely normal gastric mucosa. Gastric biopsy showed lymphoid aggregates without Helicobacter pylori infection. Serologic testing demonstrated a positive IgG and IgM antibodies to viral capsid antigen and IgG-antibodies to EBV nuclear antigen 1.Serology for cytomegalovirus,herpes simplex and parvovirus B19 were negative. The present case illustrates the broad spectrum of EBV disease and that it should be regarded as a cause of gastrointestinal lesions.

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Gastroenterology & Nutrition

Satisfactory Switch to Infliximab after Adalimumab Loss of Response in a Teenager with Severe Crohn`s Disease

Andreia Florina Nita ^1,2 , Gabriel Becheanu ^2,3 , Daniela Pacurar ^1,2

¹ Paediatrics, Grigore Alexandrescu Emergency Hospital for Children, Romania

² Paediatrics, Carol Davila University of Medicine and Pharmacy, Romania

³ Histopathology, Victor Babes Clinical Hospital for Infectious and Tropical Diseases, Romania

Introduction: Paediatric inflammatory bowel disease is often more aggressive compared to adults and the current tendency is early use of monoclonal antibodies in severe cases, loss of response being the therapeutic challenge.

We present the case of a teenager with Crohn`s Disease, naive to anti-TNF therapy who developed antibodies to Adalimumab and was switched to Infliximab.

Case Presentation: A 13 years old teenager was admitted for alopecia areata, nummular eczema, abdominal pain and weight loss (17 kg) lasting for three months. His mother reported repeated episodes of diarrhea but he denied.

He was diagnosed with Crohn`s Disease and Pediatric Crohn`s Disease Activity Index score was 60, reflecting a severe disease activity. He was started on exclusive enteral nutrition as induction therapy with a good response. We decided to introduce azathioprine not steroids(CS) based on his reluctance to receive CS due to adverse effects. After six months he presented bloody stools and after nine months he developed anal abscess and perianal fistulae which required surgical intervention. Anti-TNF agent Adalimumab was introduced in the treatment plan. After the first 3 months, the seric level of Adalimumab was low and he developed antibodies to Adalimumab accompanying symptoms. We stopped Adalimumab and initiated treatment with Infliximab which he has been receiving for 20 months now with two relapses addressed by dose increase and rhythm adjustment.

Discussions and Conclusion: In naïve patients loss of response to Adalimumab is expected to be rarer. A loss of response to Infliximab may affect the response to subsequent Adalimumab treatment based on cross-reactivity of antibodies even though the two drugs are different idiothypes. However, there is a general lack of experience on the use of Infliximab after Adalimumab failure.

In Crohn`s Disease treatment strategy, Infliximab might be a solution after Adalimumab loss of response due to Adalimumab antibodies development.

945

Gastroenterology & Nutrition

What about a Negative Serology in an Adolescent with Celiac Disease

Case Report

Joanna Ashworth¹, Carolina Fleming², José Ramon Vizcaíno², Gisela Silva³, Helena Silva³, Esmeralda Cleto⁴, Marta Tavares³, Rosa Lima³

¹ Pediatric Department, Centro Materno Infantil do Norte, CHUP, Portugal

² Anatomic Pathology Service, Pathology Department, CHUP, Portugal

³ Pediatric Gastroenterology Department, Centro Materno Infantil do Norte, CHUP, Portugal

⁴ Pediatric Hematology Department, Centro Materno Infantil do Norte, CHUP, Portugal

Background: Celiac disease (CD) is an immune-mediated systemic disorder elicited by gluten. Classic diagnosis is based on positive tissue transglutaminase type 2 (TTG2) antibodies and histopathological features as villi atrophy. Seronegative celiac patients are very rare in Pediatrics. We describe a unique case of celiac disease in a seronegative adolescent.

Case Report: A 15-year-old girl diagnosed with Blackfan-Diamond anemia at birth was referred to a Pediatric Gastroenterologist due to abdominal pain that started two months prior. She was on immunosuppressive therapy with systemic corticosteroids due to her hematological disease up to three months prior. The pain was more intense in the right upper quadrant and the diagnosis of primary biliary dyskinesia was proposed. Celiac screening workup (TTG2, Gliadin and endomisium antibodies) was negative. During an intense pain episode she was admitted to hospital and underwent an upper endoscopy which showed no relevant features. Gastric and duodenal samples were taken and microscopical examination showed moderate villi atrophy with intraepithelial lymphocytosis corresponding to a grade 3b in the modified Marsh classification (Marsh-Oberhuber). Clinical remission was achieved after a gluten-free diet (GFD). One year after diagnosis duodenal/bulb biopsy was repeated showing marked improvement without villi atrophy or crypt hyperplasia but with some intraepithelial lymphocytes (less than 30 per 100 enterocytes) which corresponds to a grade 0/1 in the modified Marsh classification (Marsh-Oberhuber). She remains clinically well on a GFD.

Conclusions: CD antibodies are not detectable in the blood of all patients with DC, especially in those under systemic immunosuppression. Strong clinical suspicion should always lead to further investigation with histolopathological analysis even in cases with negative serology.

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Gastroenterology & Nutrition

No Longer an Adult Disease

Ruxandra Simona Bacanu ¹

¹ Pediatrics, General Hospital Tomelloso, Spain

Background: When caring for children, we are all prepared for evaluating abdominal pain as it seems like every condition leads can cause belly pain.

Aim of Paper: To demonstrate that we have to remain vigilant for those subtle presentations of abdominal pain of more omnious conditions.

Material and Method: We present the case of a 8 years girl we arrived to Emergency Room with a 36 hours history of intensive abdominal pain and vomiting. En ER was realized urgent blood tests which revealed hypertransaminasemia and elevated total bilirubin on account of direct bilirubin. The girl had no history of any moment of posible contagion, signs of infection or other sympotms which could explain a frequent etiology of cytolysis in children.

The physical examination revealed an overweight child with an abundante abdominal pannus, with difuse abdominal pain, predominantly in the epigastrum.

We proceed to an abdominal ultrasonography which revealed a gallbladder with multiple stones inside and a discreet dilatation of coledocian.

Due to these findings, we iniciated medical treatment with antibiotics, with improvement of clinical and analitical tests in 24 hours.

Discussions: Cholelithiasis is being recognized in the children with increasing frequency. In children, there may be any number of possible etiologies for the development of gallstones. In the majority, specific etiologic factor is never established. In our case, we suppose the overweight is directly implicated in the produce of gallstones. Differentiating gallbladder disease in children from other causes of abdominal pain can be very difficult, but probably the single most important factor aiding in the diagnosis is simply main.- taining high index of suspicion that it can and does occur in children.

Conclusions: Not all kid belly pain is benign. Think of cholecystitis, even in kids.

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Gastroenterology & Nutrition

Helicobacter Pylori Infection and Recurrent Abdominal Pain in Children

Ruxandra Simona Bacanu ¹ , Mauricio Moreno López ¹

¹ Pediatrics, General Hospital Tomelloso, Spain

Background: Helicobacter pylori (HP) is a gram-negative bacillus responsible for one of the most common infections found in humans worldwide. The incidence is 3-10% of the population each year in developing countries compared with 0.5% in developed countries. Most children infected with H pylori are asymptomatic. The relationship between H pylori infection and recurrent abdominal pain (RAP) is controversial.

Objective: To determine the incidence of HP infection in children with RAP.

Material and Method: The study is a retrospective one. We have taken in the study all the children with recurrent abdominal pain caused by HP infection that came as “new outpatient” in Pediatrics, between January 2017 – December 2018 and we analized the management they received.

Results: From a total of 1004 “new outpatients” with RAP, 37 were confirmed with HP infection (3.68%), a higher rate of infecion than we found out in different studies. In all the pacients, the first tratment was represented by OAC asociation, with a 67.56% rate of cure (25 pacients). From the other 12 cases, 9 pacients cured after the second tratment (OMA asociation). Two more patients remained asintomatics after the second tratment. We confirmed antibiotic resistence in only 1 case (both metronidazole and claritromicin) and the pacient became asintomatic and cured with OAR asociation.With the two “clasical” tratments, for 14 days, we obtained the cure in 91.9% patients.

Conclusion: The incidence in our area is not concordante with other studies; that could be explained by the mixture of population here. Despite of this, we obtained very good results with the clasical treatment.

1056

Hepatic Glycogenosis: About a Series of 15 Cases

Naima Baddouh ¹ , S. Mrhar ¹ , N. Hazzab ¹ , A. Daoudi ¹ , H. Nassih ¹ , A. Bourrahouat ¹ , I. Ait Sab ¹

¹ Pediatrics B Department, Mother and Child Hospital, Mohammed VI University Hospital Faculty of Medicine, Cadi Ayyad University, Morocco

Hepatic glycogenosis are a group of inherited disorders characterized by enzyme defects that affect the glycogen synthesis and degradation cycle.

Objective: To describe the characteristics of patients with hepatic glycogenosis, the presentation types and to highlight the diagnostic and therapeutic difficulties in our population.

Methods: The study group consisted of 15 patients admitted in hospital between January 2012 and December 2018.

Results: 11 boys and 4 girls. The sex ratio was 2.75. Ten patients had type I glycogenosis, four patients had type III and one had type XI. The consanguinity of the parents was found in 11 patients. The referral reason was growth failure in 11 patients and hypoglycemia in 7 cases. Hepatomegaly was present in 13 cases. 5 patients had a facial dysmorphism. Biologically, hepatic cytolysis was present in 10 patients and hypertriglyceridemia in 7. Liver function was normal in all patients. Hepatic biopsy was performed in all patients and showed histological signs of glycogenosis. Therapeutic management was based on dietary measures. All patients are alive. 3 cases have progressed to cirrhosis.

Conclusion: Hepatic glycogenosis have heterogeneous clinical manifestation. It has to be kept in mind whenever important hepatomegaly and/or hypoglycemia are present.

1057

Fanconi-Bickel Syndrome: Report of a Case

Naima Baddouh ¹ , A. Ouayad ¹ , R. Kouta ¹ , H. Nassih ¹ , A. Bourrahouat ¹ , I. Ait Sab ¹

¹ Pediatrics B Department, Mother and Child Hospital, Mohammed VI University Hospital Faculty of Medicine, Morocco

Fanconi-Bickel syndrome (SFB) is a rare glycogenosis characterized by glycogen accumulation in the liver and kidneys, severe proximal renal tubulopathy, and abnormal glucose and galactose metabolism.

Case presentation: We report the case of a 5-year-old girl from a non-consanguineous marriage, who presented for growth failure and abdominal distention that progressed gradually from birth. The clinical examination showed a "doll-like" face, a size at -4DS, signs of rickets and hepatomegaly. Biological assessment showed hypoglycemia, hypophosphoremia, normal calcium rate, elevated PAL rate, normal transaminases and TP, hypercholesterolemia and hypertriglyceridemia, metabolic acidosis. In urine analysis we found glycosuria, ketonuria, amino aciduria and phosphaturia. X-rays showed diffuse osteopenia with a typical appearance of rickets. Liver biopsy showed glycogen accumulation in the hepatocytes. The treatment consisted of phosphorus and vitamin D supplementation with low galactose diet and split meals.

Conclusion: This report describes the clinical history of an affected child and highlights the importance of continued follow-up in order to extend our understanding of the history of this rare metabolic disorder.

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P1: Gastroenterology & Nutrition 1 (Friday, September 20, 2019 18:15)

Gastroenterology & Nutrition

Food Parenting Practices and Obstacles: What are Parents of Preschool Children doing to promote their Child’s Healthy Eating?

Luisa Barros ¹ , Cátia Alves ¹ , Patricia Nogueira ¹ , Eugénia Oliveira ¹ , Ana Isabel Pereira ¹

¹ Faculdade de Psicologia, Universidade de Lisboa, Portugal

Background: Eating habits develop early in childhood, and tend to persist into adulthood. Parents play a key role in the development of children`s eating habits. Although most parents are motivated to help their young children eat a healthy diet, their parenting food practices and the perception of barriers may undermine the reaching of these goals. As such, it is critical to develop comprehensive and culturally valid measures of these critical dimensions of food parenting.

Objective: A sequence of two studies aimed to identify food parenting practices and obstacles most reported by parents of Portuguese preschool children, and to develop two validated and culturally adequate questionnaires to be used in future intervention studies.

Methods: Firstly, a semi-structured interview was conducted with 12 mothers of preschool children. Interviews were recorded and transcribed and its contents analyzed according to Thematic Analysis. Secondly, two questionnaires were developed, based on the interview results, relevant literature and previous food parenting instruments; 200 parents of 2 to 6 years old children filled the questionnaire online.

Results: Parenting practices included items related to restriction, pressure to eat, food as reward, permissiveness, restriction of junk food, encouraging fruits and vegetables intake, modeling, negotiation, and self-regulation strategies. Barriers were related to the child’s characteristics, parent’s characteristics and the context. Two instruments were developed and tested, showing good psychometric properties. Parents of preschool children reported using simultaneously food effective and ineffective parenting practices. Parents reported using more often modeling and self-regulation promotion but also restriction. All barriers were considered moderately important with parent-related barriers considered more important.

Conclusion: Results point to the relevance of advancing our understanding of parenting food practices and difficulties to better tailor preventive interventions to promote healthy eating habits during early childhood.

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Gastroenterology & Nutrition

The Impact of a Short-Term Intervention on Adolescent Eating Habits and Food Knowledge

Tal David Berger ¹ , Anna Gorodnichenko ¹ , Akiva Fradkin ^1,2 , Batia Weiss ^1,2

¹ Division of Pediatric Gastroenterology and Nutrition, Edmond and Lily Safra Children's Hospital, Tel- Hashomer, Israel

² Sackler Faculty of Medicine, Tel- Aviv University, Israel

Objective: To evaluate the effect of school-based intervention on the nutritional knowledge, eating habits and physical activity of high school students.

Methods: A prospective questionnaire-based study. Students of the 8-11^th grade from one high school in Israel prospectively fulfilled anonymous, previously validated, questionnaires at the beginning of the academic year (September 2014). During the following year, vending machines containing milk products were installed within the school facility, and the students were given 2 informative nutrition lectures regarding proper nutrition for age, calcium requirement and importance, and physical activity. In addition, one active sport-day was performed. At the beginning of the following academic year (September 2015), the students fulfilled the same questionnaires again. We analyzed the results for the whole study group and according to students` age: 13-15 years (Group I) and age 16-18 years (Group II).

Results: 330 teenagers, age 15.1 ± 1.39 years, 56% males, were included. Response rate was 83.6%±0.4% to multiple-choice questions, 60.7%±0.5% to multiple section tables, and 80.3%±0.9% to open questions. Post-intervention there was an increase in the habit of eating breakfast (57.0% vs. 47.5%, p=0.02) and a decrease in purchasing food at school (61.6% vs 54.3%, p=0.003). No changes were observed in consumption of milk products, knowledge regarding calcium and vegetable consumption, or sport activities.

Conclusion: A combination of active and passive nutrition-oriented intervention at high school resulted in a significant change in part of the eating habits, but nutritional knowledge and physical activity remained unchanged.

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Gastroenterology & Nutrition

Malnutrition in Children of Northern Mozambique: Only a Small Picture of a Global Reality!

Joana Caldeira Santos ¹ , Sofia Simões Ferreira ¹ , Andreia Teles ¹ , Mulaja Étienne ² , Carla Rêgo ³

¹ Department of Pediatrics, Vila Nova de Gaia/Espinho Hospital Center, Portugal

² Department of Pediatrics, Central Hospital of Nampula, Mozambique

³ Department of Pediatrics, CUF Hospital. CINTESIS - Faculty of Medicin of Porto University. Catholic University of Portugal. Health4MOZ ONGD, Portugal

Background: Malnutrition remains a major cause of morbimortality in Mozambique, being related to 45% of deaths among children under five years.

Objective: Characterize nutritional status, comorbidities, dietary pattern and clinical evolution of children hospitalized for undernutrition.

Methods: Prospective study conducted in a Central Hospital in northern Mozambique (Nampula) in August and September 2017. WHO criteria were used to characterize nutritional status and a questionnaire was applied.

Results: 61 children were hospitalized in malnutrition sector, 16.4% with wasting and 83.6% with both stunting and wasting, which was severe in 78.7%, predominantly marasmus (55.7%). The median age was 17 (IQR 11-25) months, 50.8% were male. The majority of mothers breastfed their children (94.7%) for a mean of 12±7 months. Half of parents only spoke Emakhuwa (local language), the majority (53.1%) were Muslim, unemployment in both parents was present in 45.9% and 34.4% reported past use of Mozambican witchdoctors. The majority had neither piped water supply (80.3%) nor electricity (72.1%). They reported a median of 3 meals each day, based predominantly on roots. The most frequent associated conditions were anemia (96.4%), AIDS (31.1%) and malaria (26.2%). There was an association between AIDS and stunting (p=0.023). Severe malnutrition was associated with younger age (p=0.008) and less consumption of fruit and vegetables with marasmus (p=0.004 and p=0.032, respectively). Hospitalization had a median of 15 days, but 13.1% of children left the hospital with their parents without medical discharge and other 13.1% died, mostly as consequence of dehydration/hypovolemic shock.

Conclusion: We emphasize the young age, the severe presentation and the high morbimortality associated with undernutrition, as well as the great prevalence of HIV/AIDS and anemia. Lack of clean water and sanitation in addition to dietary deficiencies are main problems. The high rate of hospital abandonment and witchdoctors attendance reflects the need for health education in Mozambique.

525

Hemato – Oncology

Accidental PEComa: Case Report of a Rare Entity

Maria Filomena Cardosa ¹ , Sofia Lima ² , Maria Knoblich ² , Isabel Afonso ³ , Cristina Moreira ⁴

¹ Serviço de Pediatria, Hospital Garcia de Orta, Portugal

² Serviço de Cirurgia Pediátrica, Hospital Dona Estefânia – Centro Hospitalar Universitário de Lisboa Central, Portugal

³ Unidade de Gastroenterologia e Hepatologia Infantil, Hospital Dona Estefânia – Centro Hospitalar Universitário de Lisboa Central, Portugal

⁴ Serviço de Oncologia Pediátrica, Instituto Português de Oncologia, Portugal

A previously healthy girl was referred to the paediatric gastroenterology consultation at the age of four due to recurrent umbilical pain. During workup, an abdominal ultrasound (US) revealed two gallstones with 4 mm of diameter without dilation of the biliary tract. She remained in follow-up, asymptomatic, no physical findings and stable US. At the age of ten, a supra-umbilical mass was detected on US, and MRI revealed an umbilical intra-peritoneal tumor (45x40x45mm) with well-defined limits, heterogeneous, predominantly solid, highly vascularized, and compressing inferior vena cava without local invasion. Surgical mass resection was performed, and histology revealed morphological and immunohistochemical aspects of a PEComa of the abdominal wall. Given the size of the lesion ( 5 cm) and admitting uncertain malignant potential, she was referred to the pediatric oncology center and submitted to margin enlargement. Three years later, she remains asymptomatic without clinical or imagiological evidence of relapse.

Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor with myomelanocytic differentiation. It mainly occurs in middle aged females and can be found at any location, being kidney the most common. The differential diagnosis of PEComas is fairly broad but the immunohistochemical coexpression of melanocytic (HMB45) and smooth muscle (actin and desmin) biomarkers make the diagnosis. They may behave indolently but entail variable malignant potential. When there isn’t evidence of metastasis, surgical resection is the recommended approach. The benefits of chemotherapy or radiation haven’t been established thus far but some clinical reports show good results with mTOR inhibitors.

With this case we intend to recall the importance of considering this rare etiology in the evaluation of a mesenchymal tumor and enhance the importance of immunohistochemical biomarkers for the definitive diagnosis. The correct histological classification is essential to assess the potential of malignancy and thus assure the most appropriate therapeutic approach.

175

Gastroenterology & Nutrition

Administration of Probiotics Enriched Gut Bacteria Diversity and Glycan Degrading Ability in Small Bowel Transplantees

Pi-Feng Chang ¹ , Yu-Cheng Lin ¹ , Kevin Liu ¹ , Yun Chen ^1,2

¹ Pediatrics and Electronic Engineering, Far Eastern Memorial Hospital and Oriental Institute of Technology, Taiwan

² Pediatric Surgery, Far Eastern Memorial Hospital, Taiwan

Background: The role of probiotics among small bowel transplantation (SBT) patients is intriguing because bacteria reside in the transplanted organ per se and hosts’ immune functions are inevitably compromised. Glycans are the primary nutritional source for microbes in the human gut. Analysis of glycan degradation by the gut microbiota is an important tool for advancing our understanding of bacterial metabolism in the gut.

Objectives: The aim of this study was to quantify glycan processing capabilities and investigate the relationship between the changes of gut microbiota and glycan metabolism before and after probiotics treatment.

Methods: The SBT patients without obvious sepsis, weaning from parenteral nutrition (PN) and normal oral intake were enrolled in this study. During the study period, they were receiving standard doses of Tacrolimus and steroids. These SBT patients received oral probiotics (CBM588: Clostridium butyricum MIYAIRI 588, 1.5 ×10⁹ CFU/day) for 1 month. Fecal samples were collected at before, 1 week and 1 month after oral probiotics therapy. Next-generation sequencing targeting 16S ribosomal sequences from fecal materials was used to evaluate gut microbiota and glycan degradation potency, respectively. We compare the changes of microbiota and glycan degrading capabilities before and after probiotics treatment.

Results: Significant shifts of bacteria compositions were found after one month of CBM588 ingestions. Specifically at the family level, Bacteroidaceae increased with a substantial boost from 4% to 21% but Enterobacteriaceae decreased by a large margin from 41% to 34%. Functional inference with 16S-based microbiome extrapolations suggested characteristic enhancement of glycan degrading capabilities with CBM588 ingestions. Metagenomes from shotgun sequencing confirmed the conjecture, showing both diversified capabilities and increased quantities of carbohydrate (glycan)-active genes. The improvements spread beyond the enzyme categories directly offered by the CBM588 genome.

Conclusions: In sum, CBM588-enhanced glycan-processing capabilities well exemplified a metabolic driver potential of probiotics upon the bacteria ecosystem in human bowels.

965

Gastroenterology & Nutrition

Meckel`s diverticulum: a rare case of rectal bleeding in a teenager

Ines Coelho ¹ , Sofia Baptista ¹ , Teresa Monteiro ¹ , Rita Justo Pereira ¹ , Daniel Tiago ¹ , Stefanie Pereira ²

¹ Pediatrics, Centro Hospitalar Universitário do Algarve, Unidade de Faro, Portugal ² Pediatric surgery, Centro Hospitalar Universitário do Algarve, Unidade de Faro, Portugal

Introduction: Rectal bleeding is a common problem in pediatrics and in the majority of cases the causes are transient, benign and simplytreated. The age of onset relates to certain diagnoses: anal fissures and intussusception in infant, and lymphoid hyperplasia and inflammatory bowel disease in the older child. In children younger than 2 years of age we also have to think in Meckel`s diverticulum (MD). In these cases the majority of patients don’t have symptoms, but the ones who are symptomatic, often present rectal bleeding, which is usually painless.

Case Report: A 12 year-old-boy was admitted to the paediatric emergency room with a 3-day history of abdominal pain with rectal bleeding. No relevant personal or family history. On examination, he presented a BMI 95^thpercentile, he was pale, but was hemodynamically stable, with no pain on abdominal palpation. The hemogram showed an hemoglobin of 7 g/dL, so he received a blood transfusion and was admitted to the ward. Throughout his stay he did an upper and lower endoscopy and an MR enterography, which didn’t reveal any alteration. We were suspicious of an MD and after having done a technetium-99m pertechnetate scan, the diagnosis was confirmed. He underwent surgical resection of the MD (segmental intestinal enterectomy), which ran without intercurrences. The postoperative period was uneventful and he was discharged on day 6, clinically well.

Conclusion: Meckel`s diverticulum is a common congenital gastrointestinal malformation in children but is rare in teenagers. It has various clinical presentations and it is necessary to maintain a high suspicion in the paediatric age group. Serious complications rarely happen but when they do, they can cause consequences, such as mechanical obstruction, intestinal perforation and shock. Thus, prompt diagnosis and proper treatment are essential in warranting a better outcome.

402

Gastroenterology & Nutrition

Usefulness of Oesophageal Length Measurement in Paediatric Gastroscopy

David Croaker¹, Himasha Nanayakkara²

¹ Department of Paediatric Surgery, The Canberra Hospital, Australia

² Medical School, University of Notre Dame, Sydney, Australia

Background: The senior author has had a routine of including an oesophageal length measurement routinely at paediatric endoscopy. The authors hypothesise that a short oesophagus predicts reflux changes at pathology.

Aim: This audit seeks to assess the utility of oesophageal length measurement.

Methods: The study is a retrospective chart review. At gastroscopy the distance from the teeth to the squamo-columnar junction is noted. Demographics, oesophageal length, clinical background and pathology data were collected and analysed in an Excel spreadsheet.

Results: 1143 endoscopy reports were reviewed. 823 oesophageal length measurements were available. Mean oesophageal length at 12 months is 21.5 cm, and at 10 years is 33.2 cm. The line of best fit has oesophageal growth ~ 0.1 cm per month from 0 to 16 years. Some degree of inflammation was noted in 313 of 877 pathology reports. The longest and shortest oesophageal length quartiles normalised for height had similar rates of reflux changes on pathology. As expected oesophageal, length in TOF patients is short, and oesophageal growth slower in TOF than in others. A modest tendency for eosinophilic oesophagitis to correlate with short oesophagus was noted (length difference ~ 0.7cm; p = 0.015).

Discussion: Oesophageal length measurements normalised for both height and age failed to correlate with reflux changes which were nonetheless widespread in TOF patients. There was otherwise no strong relationship between oesophageal length and reflux changes in the general population.

Conclusions: While our measurements seem valid and accurate, their usefulness in predicting reflux pathology in the general population is limited.

690

Gastroenterology & Nutrition

Is Bile in the Stomach Related to Paediatric Chronic Abdominal Pain?

David Croaker¹, Himasha Nannayakkara²

¹ Department of Paediatrics and Child Health, The Canberra Hospital, Australia

² Medical School, Notre Dame University, Australia

Background/Aim: While bilious vomiting is regarded as a worrying symptom and sign in acute paediatric surgery, chronic abdominal pain is a common non-acute reason for referral for endoscopy. Chronic non-specific gastritis is often reported on biopsy in our patients. The authors asked how often bile was seen in the stomach on gastroscopy, and asked whether this correlated with gastric pathology to support the notion of bile gastritis as a cause of chronic abdominal pain in children.

Methods: Retrospective chart review of a paediatric gastroscopy practice. Routine biopsies are usually taken from gastric antrum. Gastric contents were noted. Only patients with recorded gastric contents were included. The pathology report was reviewed. Data were stored in Excel. Presence of gastric bile was compared against histology and presentation. Proportions were compared by Fisher exact test.

Results: 769 patients with available data on the gastric contents were reviewed, 743 of whom had histopathology reports. Results are summarised in the table. These proportions were not significantly different by Fischer Exact test. Patients with bile stained gastric contents however were nearly twice as likely to have been referred for investigation of abdominal pain as those with no bile staining. (16% vs 9% p=0.007 by Fisher exact test.) No relationship to distal oesophageal reflux changes was found to explain the pain correlation.

Conclusions: While bilious vomiting remains a red flag of the greatest importance, the presence of bile in the stomach without vomiting has no clear relation to gastric histopathology, but may be associated with abdominal pain.

698

Gastroenterology & Nutrition

Alagille Syndrome – The Difficulty of Initial Diagnosis

Ruxandra Darie ¹ , Raluca Maria Vlad ^1,2 , Irina Dijmarescu ^1,2 , Daniela Pacurar ^1,2

¹ Paediatrics Department, “Grigore Alexandrescu” Emergency Children’s Hospital, Bucharest, Romania

² Paediatrics Department, “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania

Background: Alagille syndrome is a rare autosomal dominant disorder with systemic manifestations: paucity of the bile ducts, abnormalities of the heart, eye, skeleton and kidneys. It is diagnosed in children presenting with jaundice in the first 6 months of life (70%) or cardiac murmur (17%).

Objective: This case presentation emphasises the challenges faced in terms of diagnosis and therapy for one of these patients.

Method and Results: We report the case of a 3 days old boy admitted in the Paediatrics Department of “Grigore Alexandrescu” Children’s Hospital, Bucharest for bilious vomiting and intense jaundice. The newborn hadn’t pass the meconium. Clinical examination showed abdominal distension and cardiac murmur. Laboratory tests revealed high levels of conjugated bilirubin, normal transaminases, serum proteins and blood coagulation. Viral serologies were all negative. Thoraco-lumbar X-ray of the spine didn’t reveal any anomalies. Echocardiography described large pulmonary stenosis. Barium enema failed to pass meconium, exploratory laparotomy was performed for deobstruction. Cystic fibrosis was excluded. Alagille syndrome was the main diagnostic considered, but liver biopsy failed to reveal paucity of the bile ducts. Over the next two years the pacient underwent extensive investigations. A posterior embryotoxon was described. Abdominal CT could not visualise the bile duct. Abdominal MRI + cholangio-MRI showed thin intra/extrahepatic biliary ducts, cirrhotic nodules, the gallblader was not visible. Genetic testing was not available. The pacient associated failure to thrive. Signs of chronic disease began to surface in time: severe jaundice, hepatosplenomegaly, abdominal collateral circulation, persistent hepatic cytolysis and cholestasis, dyslipidemia. Due to progressive chronic hepato-biliary disease and advancing cirrhosis in a 2 years old with failure to thrive, liver transplant is now considered.

Conclusion: Although Alagille should be a straight forward diagnosis, in certain instances it takes time and sustained efforts to reach it and set the course for an optimal therapeutic approach.

812

Gastroenterology & Nutrition

Autoimmune Overlap Syndrome in A 4 Year Old Girl – A Case Report

Irina Dijmarescu ^1,2 , Gabriela Mitea ¹ , Alexandra Coroleuca ¹ , Daniela Pacurar ^1,2

¹ Pediatrics, "Grigore Alexandrescu" Emergency Hospital for Children, Romania

² Pediatrics, "Carol Davila" University of Medicine and Pharmacy, Romania

Background: The overlap syndrome is characterized by the simultaneous occurrence of more than one autoimmune disease.

Objective: We aimed to present the difficulties encountered in diagnosing and treating a young child with an overlap syndrome.

Case report: We present the case of a 4 year old female patient who was diagnosed with autoimmune hepatitis, primary sclerotic cholangitis and ulcerative colitis. The patient was being investigated for loss of appetite, weight loss, hepatosplenomegaly and bloody stools. The onset was 5 months prior to the diagnosis with bloody stools. Infectious causes were considered and the patient was treated accordingly. She had recurrent fever and hepatosplenomegaly. Laboratory tests showed severe anemia, high transaminases, cholestasis, high inflammatory markers, hypergammaglobulinemia (50,8 g/dl) and high fecal calprotectine (1370 mcg/g). Viral causes of liver cytolysis were excluded. Due to the persistence of liver cytolysis further investigations were performed and LKM1 antibodies were positive. At the time of the diagnosis the child was completely nonimmunized. Subsequently she received first doses of all mandatory vaccines but no following doses. The colonoscopy showed rectal mucosa with hyperemic patches, histopathological examination being consistent with ulcerative colitis. The liver biopsy displayed advanced hepatic fibrosis and cholangitis. Thus, correlating all data, the diagnostic of an overlap syndrome (autoimmune hepatitis and primary sclerotic cholangitis) associated with ulcerative colitis was established. She received systemic corticotherapy, tapered afterwards (complete exclusion was not possible due to recurrence of bloody stools and liver cytolysis) and azathioprine since the time of diagnosis.

Conclusions: Overlap syndrome is a possibility that should be considered in patients with autoimmune diseases. Further investigations should be conducted in order to establish a complete diagnosis of these patients in order to treat them accordingly.

762

Gastroenterology & Nutrition

Efficiency and Safety of GABA Derivatives for Treatment of Functional Dyspepsia in Children

Anna Drokh ¹ , Marina Mamenko ² , Ivanna Romankevych ³ , Katerina Boyarska ⁴ , Hanna Kozhyna ⁵ , Raisa Moiseienko ⁶

¹ Shupyk National Medical Academy of Postgraduate Education, PhD student, MD, Ukraine

² Shupyk National Medical Academy of Postgraduate Education, Professor, Dean of Pediatric Faculty, MD, PhD, DMs, Ukraine

³ Shupyk National Medical Academy of Postgraduate Education, Assistant Professor of CEMSIT, PhD, MD, Ukraine

⁴ Kyiv City Children's Clinical Hospital, Head of the Department of General Pediatrics, MD, Ukraine

⁵ Kyiv City Children's Clinical Hospital, MD, Ukraine

⁶ Shupyk National Medical Academy of Postgraduate Education, Professor, Vice-Rector for Clinical Work, MD, PhD, DMs, Ukraine

Background and Aims: The quality of life (QL) in children with functional dyspepsia (FD) is often impaired due to the psychological disorders and cognitive dysfunction seen in these patients. The aim of this study was to evaluate the efficacy and safety of phenibut in children with FD.

Methods: 40 children with FD were randomized to 1 month of symptomatic treatment+phenibut (n=20, 400 mg/d 4 times per day according to the instructions) or exclusively symptomatic treatment (n=20, Rome IV). QL was determined by a questionnaire “PedsQL”. The memory was evaluated by method of "Memory for Numbers" and "RAM". The attention was determined by the method of "Proofreading" and "Technique of Münstenberg".

Results: At 1 month, the active group demonstrated: the improvement of physical functioning (p=0.007) and vs control group (p=0.043): increasing of the emotional functioning (p=0.043) and vs the control (p=0.033); getting better in the functioning at the school (p=0.016) and vs the control (p=0.045). The phenibut group reported the increasing of the level of selective attention (p=0.023) and vs control group (p=0.041). The productivity and consistency of attention in the phenibut group was higher after treatment (p=0.001) and vs control (p=0.009). The level of short-term visual memory in the active group improved significantly after treatment (p=0.047) and vs control (p=0.038). RAM in the phenibut group was higher after treatment (p=0.007) and vs control group (p=0.013).

Conclusions: Phenibut was associated with appropriate therapeutic response in cognitive function and QL in children with FD. It was safe and well tolerated in used dosage.

604

Gastroenterology & Nutrition

Tandem Breastfeeding and Human Milk Macronutrients Content

Gilad Rosenberg ¹ , Dror Mandel ^2,3 , Ronella Marom ^2,3 , Laurence Mangel ² , Ronit Lubetzky ^1,3

¹ Pediatrics, Dana Dwek Children's Hospital, Tel Aviv Medical Center, Israel

² Neonatology, Dana Dwek Children's Hospital, Tel Aviv Medical Center, Israel

³ Sackler Faculty of Medicine, Tel Aviv University, Israel

Introduction: Little is known about the effect of tandem breastfeeding (the practice of breastfeeding two different age siblings) on human milk (HM) macronutrients content. We aimed to study macronutrients and energy content in HM collected during tandem breastfeeding.

Methods: Prospective-observational study. Mothers who breastfed their infants during pregnancy and intended to continue breastfeeding after delivery were recruited (tandem-group) and compared to a group of mothers who did-not breastfed during pregnancy, gave birth and breastfed one infant (control-group). Milk was collected during the last 4-weeks of pregnancy (tandem-group only), at 72-hours (colostrum, both groups) and at 30-days after delivery (mature-HM, both groups). Analysis was by infrared-transmission-spectroscopy.

Results: Fifty healthy-mothers were enrolled, out of which eighteen continued breastfeeding during term-pregnancy and after labor. They were compared to data from 118 mothers who did-not breastfeed during pregnancy and breastfed only one term-infant. HM during pregnancy had significantly lower fat content compared to HM in colostrum and mature-milk from the same mother (2.5±1.4 vs. 2.9±0.9, p=0.003; 2.5±1.4 vs. 4.2±1.2, p=0.009, respectively). Colostrum and mature-milk Fat did-not differ between tandem and controls. Protein during pregnancy was significantly higher compared to colostrum and mature-HM (3.5±1.4 vs. 2.0±0.8, p=0.001; 3.5±1.4 vs. 1.1±0.3, p0.0001, respectively). Colostrum and mature-HM protein did-not differ between the 2 groups. HM-lactose during pregnancy was significantly lower compared to mature-HM (6.4±1.4 vs. 8.1±0.2, p0.0001). Colostrum and mature-HM Lactose were significantly higher in tandem-group compared to controls. Energy content during pregnancy was significantly lower compared to mature-HM (63.4±11.4 vs. 71.9±10.0, p=0.047). Colostrum Energy was significantly higher in tandem-group compared to controls.

Conclusion: HM expressed during pregnancy has different macronutrients content compared to HM after regular-full-term pregnancy. After delivery, during tandem-breastfeeding, there are minor differences in lactose and energy only in colostrum. Thus, mothers who tandem-breastfeed can be reassured about the macronutrients content of their milk.

116

Gastroenterology & Nutrition

Audit of Checklist in Recurrent Abdominal Pain

Frances Enright ^1,2 , Hana Bashir ² , Dace Balode Novikoda ²

¹ Paediatric Outpatients, Mercy University Hosptal, Ireland

² Outpatients, Mercy University Hospital, Ireland

Background: Recurrent abdominal pain (RAP) is common at 30% in childhood especially females 4-6 years of age and 9-11 years. RAP is more commonly non-organic i.e. functional abdominal pain FAP. A symptom-based approach is usually used by clinicians to identify the red flag symptoms of organic disease. In a previous retrospective study in 2015, co-author Dace studied 41 patients with RAP. 63% had investigation but had no red flag symptoms.

A checklist with sub-typing (attached) was developed to be used by the same doctor at every consult for FAP.

Objective: Reduce unnecessary investigations, keep approach consistent and subtype the FAP.

Methods: A retrospective cross-sectional design was used by Hana in this audit. If FAP is diagnosed the subtype is filled out. The treatment plan signed by parents is filed.

Results: Hana found a total of 23 (n=23) new patients with recurrent abdominal pain who had the checklist filled. One case (4.3%) had investigations in the absence of red flag symptoms. Six cases (26%) reported no red flag symptoms and did not undergo any investigation. Sixteen of the twenty-three cases (70%) reported at least one red flag symptom and received agreed investigations.

3 of the 23 children had organic causes (Gastritis, reflux and Crohns). The subtypes of FAP we identified were 5% (1) functional dyspepsia (upper abdomen discomfort), 50% (10) irritable bowel (FAP with alteration in stool consistency), 5% (1) abdominal migraine (history of maternal migraine and child has paroxysmal episodes of FAP with associated anorexia, pallor, nausea) and 20% (4) Functional abdominal pain syndrome (by exclusion).

Conclusion: This audit illustrates a significant reduction (63% to 4.3%) in the frequency of unnecessary investigations performed in the absence of red flag symptoms following the introduction of the checklist. Irritable bowel functional abdominal pain is commonest subtype. We recommend this method.

209

Gastroenterology & Nutrition

Hypertrophyc Pyloric Stenosis: A Radiographic Finding

Celia Fabra Garrido ¹ , Marta Dorado Criado ¹ , Ursino Adrián Barrios Machain ¹ , Paloma López Ortego ²

¹ Pediatrics, Hospital Universitario La Paz, Spain

² Neonatology, Hospital Universitario La Paz, Spain

Introduction: Hypertrophic pyloric stenosis (HPS) is one of the most frequent causes of non-bilious vomiting in infants. The diagnose is usually carried out by ultrasonography and only a few cases diagnosed by simple radiography are reported in the medical literature.We present a case that was diagnosed incidentally by the “caterpillar sign”, a characteristic radiographic sign of HPS.

Clinical Case: A 2-month-old male patient was operated on for a cervical teratoma. Subsequently, he presented weakness of the oropharyngeal musculature and difficulties in swallowing as well as severe gastroesophageal reflux. He was under omeprazole, domperidone and continuous debit nutrition through a transpyloric tube. Despite the treatment, the patient was presenting frequent regurgitation and vomiting and a chest-abdomen x-ray was perfomed to check the correct position of the probe. The radiography showed a distended stomach with increased peristaltic activity and he was diagnosed of HPS (Figure 1). The diagnosis was confirmed by abdominal ultrasound (figure 2) and a submucosal pyloromyotomy was perfomed with successful evolution.

Figure 1. Chest-abdomen x-ray with notable peristaltic waves in the stomach due to HPS.

Figure 2. Abdominal ultrasound: Pyloric channel with diagnostic criteria for HPS.

Discussion: The gold standard imaging test for the diagnosis of HPS is abdominal ultrasound. If it does not provide a conclusive result, barium studies or upper gastrointestinal endoscopy are other diagnostic options.

Our patient was diagnosed incidentally by simple radiography of the abdomen by the "caterpillar sign". This sign is the result of vigorous peristaltic waves against an abrupt stop at the hypertrophic pylorus.

Conclusion: This clinical case reminds us the importance of the systematic analysis in medical imaging tests, particularly in simple radiography, as despite it being a basic diagnostic tool, it is also a great diagnostic source of information.

786

Gastroenterology & Nutrition

Celiac Disease – A 13-Year Casuistic in a Tertiary Hospital

Carolina Freitas Fernandes ¹ , Bernardo Camacho ¹ , Francisco Silva ¹ , Rute Gonçalves ¹

¹ Paediatrics, Hospital Central do Funchal, Portugal

Background: Celiac disease (CD), also known as gluten-sensitive enteropathy is an immune-mediated inflammatory disease of the small intestine caused by sensitivity to dietary gluten and related proteins in genetically predisposed individuals. The disorder occurs in 0.5 to 1 percent of the general population in most countries.

Objective: Evaluating the incidence of celiac disease in the pediatric population in a tertiary hospital.

Methods: A retrospective study was carried out through review of clinical process of the pediatric patients with confirmed diagnosis of celiac disease in a tertiary hospital since 2006 to 2018.

Results: 65 cases of celiac disease were diagnosed, with a mean age of onset at 22 months and mean age of diagnosis between 4-5 years old, with no gender predominance. In the clinical presentation, 61 patients had gastrointestinal symptoms (GI), with a deficient weight progression (N = 45); abdominal distension (N = 40) and diarrhea (N = 39). 33 patients also presented non-GI symptoms, where growth retardation (N = 25) and anemia (N = 18) were the most prevalent.

Analytically all patients were positive for IgA/IgG anti-transglutaminase antibodies and/or IgA/IgG anti-gliadin antibodies. All patients, except 3, underwent upper digestive endoscopy with biopsies, whose histopathological analysis was suggestive for celiac disease in 58 patients, being classified according to Marsh criteria. 77% of patients have a positive genetic test for HLA-DQ2.

Conclusion: The diagnosis of CD is complex, especially with atypical manifestations. The diagnosis may be established after clinical correlation between symptoms, positive serology, intestinal biopsy and response to gluten-free diet. In recent years, the prevalence of CD has increased because of the greater degree of suspicion and better accuracy of the serological tests.

646

Gastroenterology & Nutrition

Nutrition Support in Pediatric Patients with Malnutrition

Aysegul Alpcan ¹ , Serkan Tursun ¹ , Hacer Fulya Gulerman ²

¹ Department of Pediatrics, Kırıkkale University, Faculty of Medicine, Turkey

² Pediatric Gastroenterology Hepatology and Nutrition, Kırıkkale University Faculty of Medicine Pediatrics, Turkey

Introduction: Malnutrition is an inadequate or irregular intake of energy, protein and other nutrients that can cause disruption of body structure and function If gastrointestinal function is normal, but oral intake is not sufficient, the food nutritives should be supplemented via oral or enteral route with feeding tube.

Methods:Patients who were admitted to Kırıkkale University Faculty of Medicine, Department of Pediatric Gastroenterology between January, 2014 and January, 2019 were included in the study. Patient information was obtained from the records retrospectively using malnutrition and enteral nutrition diagnostic codes.

RESULTS: The study included 111patients. Of the patients, 56(50.5%) were female. Different enteral feeding products were given to the patients according to their clinical status, diagnoses, and recent weight changes. In addition to the patients diagnosed with malnutrition, 23(20.7%) had GI system disease, 43(38.7%) had neurometabolic disease, 12 (10.8%) had endocrinological disease, and 2(1.8%) had hepatobiliary disease. Nine of the patients(8.1%) were fed with percutaneous endoscopic gastrostomy. Of the 111 patients examined, 28(25,2%) were received standard products, 39(35,1%) were high fiber, 7(6,3%) were high energy, and 26(23,4%) were high energy and high-fiber, 5(4.5%) hydrolyzed nutrition product, others with special diseases received products suitable for diseases they have. 74 of the patients(66.7%) were followed up and 37 of them (33.3%) were not participate in follow up with their own requests. Patients with neurometabolic disease used the enteral nutrition product continuously. In other patients, enteral nutrition support was continued or discontinued according to nutrition status and weight gain.

Results: Enteral nutritional support is a major contributor to the prevention of malnutrition, which is an important problem in the chronic disease process, and for better outcome of the actual treatment and follow-up of the disease. It makes it easier for the physician and caregiver / family who follows the patient to cope with the disease.

741

Gastroenterology & Nutrition

An Adolescent Girl with an Extraordinary Crohn Disease

Fulya Gulerman¹, Burcu Guven¹, Cansu Celik¹, Zafer Onaran², Banu Acar Celikel³

¹ Pediatric Gastroenterology, Hepatology and Nutrition, Kirikkale University, Turkey

² Ophthalmology, Kirikkale University, Turkey

³ Pediatric Nephrology and Rheumatology, Diskapi Yildirim Beyazit Research Hospital, Turkey

Inflammatory bowel disease (IBD) is a comprehensive term used to describe diseases of the gastrointestinal tract resulting from chronic inflammatory processes. Patients with Crohn`s disease, a type of IBD, also may have extraintestinal findings. Joint symptoms and mucocutaneous involvement are the most common manifestations. Therefore, the presence of symptoms such as swollen, painful joints, aphthous stomatitis, erythema nodosum, uveitis, and pericarditis should not be avoided.

A 15-year-old girl was admitted to our outpatient clinic with complaints of severe abdominal pain, nausea, vomiting and bleeding in one eye. The patient was diagnosed with acute pancreatitis and uveitis at the first evaluation, followed by bloody diarrhea and arthritis. In endoscopy, antrum, bulbus and second segment of duodenum were seen as hyperemic, nodular and edematous. In the colonoscopy, mucosa of the terminal ileum was granular and edematous. The cecum, the ascending colon, hepatic flexure, descending colon, sigmoid colon, and rectum mucosa were hyperemic, granular in appearance (cobblestone view) and lymphoid hyperplasic in appearance. The patient was diagnosed as multisystemic Crohn`s disease. The patient was followed up with prednisolone and mesalazine treatment. At the end of one-year follow-up, the symptoms of pancreatitis improved totally, but there were no response to the treatment for uveitis and arthritis, and patient developed pericarditis and bloody diarrhea continued occasionally. Infliximab, a biological agent (tumour necrotising factor alpha, TNFa blocker), was the second choice of treatment. The expected result could not be obtained and after 6 cure treatments medication was switched to adalimumab. After 6 cycles of treatment, the patient`s symptoms were completely regressed.

In conclusion, it should be kept in mind that extraintestinal manifestations of IBD can occur in pediatric patients before the original signs of the disease occur. The management of these findings with new treatment modalities may result in more positive outcomes in the future.

780

Gastroenterology & Nutrition

All Bunged Up - A Service Evaluation of a Nurse-Led Constipation Clinic

Laura Harrison ¹ , Ranya Derrick ¹ , Furhana Hussein ¹ , Brittany Reid ¹ , Kim Gordon ¹ , Sue Stevens ¹ , Peter Sullivan ¹

¹ Paediatric Gastroenterology Department, Oxford University Hospitals, UK

Background: Constipation is extremely common in children. Up to 30% of children suffer with constipation, lasting longer than 6 months in 5%. This constipation clinic was set up to provide specialist constipation management with the aim of treating and discharging all children within 2 years. However a significant number of patients are difficult to manage and remain in the clinic beyond 2 years.

Objective: The aim of this service evaluation was to analyse the characteristics of these children for common features that would allow early identification of potentially difficult cases. Additionally, their management within the clinic was evaluated to see if any improvements would aid more rapid treatment and earlier discharge.

Methods: A search was conducted for patients who had been in the paediatric outpatient constipation clinic for greater than 2 years. Their medical notes were reviewed to identify any features of this population and their management in the clinic that could lead to their ongoing difficulties with constipation.

Results: 91 patients were included in the evaluation, ranging in age from 4 to 16. Particularly noticeable common features of these patients included soiling associated with constipation in 91.2%, withholding/avoidance of using the toilet in 46.1% and issues with compliance in 34%. Furthermore they had a significant number of changes to their management (many had over 15 medication changes) in clinic, making compliance more difficult.

Conclusion: Recommendations were made for changes to management including giving the patients constipation management plans to aid patient compliance with medication and SOS appointments for those stable on therapy who may relapse. It is clear that this particular subset of paediatric patients with constipation have ongoing difficulties despite intensive outpatient management and, as they would benefit from more individualised therapy, our ongoing work involves developing a risk assessment tool to identify them early.

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Gastroenterology & Nutrition

Parents and Physicians Perception of Children`s Eating Behavior and Actual Body Mass index in Pediatric Ambulatory Setting -Qatar

Manasik Hassan ^1,2 , Mohamed Sobhy ² , Hatim Abdulerhman ² , Ahmed Alhammadi ²

¹ Peditaric, Hamad Medcial Corporation, Qatar

² Peditaric, SIDRA Medicine, Qatar

Background and Aims: Eating behavior in children’s considered part of their growth and development; one those behaviors is picky eater. Our aim is to explore and compare parents and physcinics perception of child picky eater and to determine the correlations between their attitude and actual child BMI

Methods: A cross sectional retrospective study done at Hamad Medical Corporation in general pediatrics clinics. Survey include details of demographic, questions to explore perception of picky eater among parents and physicians, compare relation between truly parents concern and the physicians evaluation for the kids regarding feeding habits and how it is affecting the children growth

Results: A total of (136) questionnaire analyzed; mean age of children’s 5 years old. (75%) of mothers were responsible for feeding and scheduling meals. (58%) of Parents considering their children as picky eater and have concerns on their growth, poor appetite and not getting optimal vitamins from meals compere to physician`s concern (43%) p value (0.005). family concern matched low BMI among children’s (BMI

Conclusion: Our study showed that parents are more likely to identify their children as being Picky eater than physicians, there was a good correlation between parent`s perception and their child’s actual BMI.Both group shared same concerns regarding picky eater behaviors. Understanding family dynamics and mealtime interactions will help in improving parenting feeding styles

657

Gastroenterology & Nutrition

A Case Report of Acute Appendicitis caused by Enterobius Vermicularis

Georgi Ivanov ^1,2 , Nikola Kolev ¹ , Anton Tonev ¹ , Dilyan Petrov ¹ , Georgi Valentinov ¹ , Aleksandar Zlatarov ¹ , Polina Ivanova ³ , Valentin Ignatov ¹ , Krasimir Ivanov ¹

¹ Department of General and Operative Surgery, University Hospital Saint Marina, Bulgaria ² Department of Pediatric Surgery, University Hospital Saint Marina, Bulgaria ³ Medical University of Varna, Bulgaria

Background: Acute appendicitis is an acute inflammation of the vermiform appendix. It is most likely due to obstruction of the lumen of the appendix and rarely can be caused by parasites. For a long time, the association between parasitic infection of the appendix and appendectomy has been widely investigated.

Objective: To describe a rare case of Enterobious Vermicularis parasitic infection presenting as a standard appendicitis without any clinical indications of parasites. This study aims to highlight the importance of parasitic infections as a deferential diagnosis of a very common condition.

Methods: We present a case report of a 11-years old girl with acute appendicitis caused by parasites. There were no signs of parasitic infection: her eosinophils were at normal range; the CRP protein levels were normal. There were no clinical symptoms as well indicating that it was a parasitic infection of the appendinx. During the laparoscopy, in the appendix were found alive helminths from the species Enterobius Vermicularis which were supposed to be the origin of the inflammation.

Results: The results of this study indicate that Enterobious Vermicularis is a rare etiological cause of acute appendicitis. The presence of this parasite can lead to serious changes in the appendix ranging to life-threatening complications.

Conclusion: Parasites are still present inffections and may cause serious inflamation even without any clinical indications. Due to that parasitic infection should be considered in any differential diagnosis of a common appendicitis.

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Gastroenterology & Nutrition

Bone Health in Adolescents with Anorexia Nervosa: Comparison of Z score Bone Densitometry with Antropometry and Serum Leves of Osteocalcin and P1NP

Pavel Kabicek ¹ , Pavel Kabicek ¹ , Jiri Behounek ¹ , Klara Borecka ¹ , Katarina Jassova ¹ , Milan Bayer ¹

¹ Thomayer Hospital Prague - Krč, Pavel Kabicek, Czech Republic

We describe a group of 16 adolescents with anorexia nervosa (AN). We performed bone densitometry and examined serum levels of osteocalcin and N-terminal procollagen 1 (P1NP) peptide. Anthropometric data and a history of the disease were evaluated. The disease lasted for an average of 7.5 months, the age of adolescents was 14-16 years. Amenorea lasted 5-12 months, twice primary amenorrhea was present. The weight loss was 5-36 kg. Examination of sex hormones has produced findings that are consistent with central hypogonadism. Estradiol was 6 times below 18.4 pmol / L, LH was 5 times below 0.1 U / l. Densitometric examination yielded below average Z scores, in 4 girls the values ​​were below 2 sigma Z score. The most pronounced changes were in the Z score of the spine (mean -0,879), the mean Z-score of the femoral neck was -0,518, and the TOTAL Z score was - 0,66. Both osteocalcin and P1NP values were below average, with two girls achieving osteocalcin less than 2.5 percentile for a given age, and P1NP reached less than 2.5 percentile for a given age in four girls. We will monitor the densitometry ​​for 1 and 2 years after the first examination and compare it with serum osteocalcin and P1NP values and we will try to suggest a treatment scheme for secondary osteoporosis in adolescents with AN. The work is supported by a Thomayer hospital grant: MZd 2018 č.2 RVO-TN/2018: DZ 1816

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Gastroenterology & Nutrition

Developmental and Behavioral Follow-up at Age 2 Years of Preterm Children Supplemented with Docosahexaenoic and Arachidonic Acid at Age 1 Year: The Omega Tots Trial

Sarah Keim^1,2,3, Kelly Boone⁴, Krista Pattison⁵, Taniqua Ingol², Grace Pelak², Kelly Sheppard², Mary Ann Nelin⁶, Mark Klebanoff^1,3,6,7, Joseph Rausch^1,2, Keith Yeates^8,9, Abigail Norris Turner^3,10, Lynette Rogers^1,6

¹ Pediatrics, The Ohio State University College of Medicine, USA

² Center for Biobehavioral Health, Nationwide Children's Hospital, USA

³ Epidemiology, The Ohio State University College of Public Health, USA

⁴ Crane Center for Early Childhood Research and Policy, The Ohio State University, USA

⁵ Pediatrics, Pennsylvania State University College of Medicine, USA

⁶ Center for Perinatal Research, Nationwide Children's Hospital, USA

⁷ Obstetrics and Gynecology, The Ohio State University College of Medicine, USA

⁸ Alberta Children's Research Institute, University of Calgary, Canada

⁹ Hotchkiss Brain Institute, University of Calgary, Canada

¹⁰ Internal Medicine, The Ohio State University College of Medicine, USA

Background: Toddlers consume low amounts of docosahexaenoic acid (DHA) post-weaning. The Omega Tots randomized, blind, placebo-controlled trial of children born at

Objective: To determine whether daily DHA and arachidonic acid (AA) supplementation at age 1 year improves development and behavior at age 2 years among children born preterm.

Methods: Omega Tots participants were followed up at 26-35 months’ chronological age to assess longer-term outcomes. Parents remained blind to treatment assignment and completed the Developmental Profile-3 (global cognitive); MacArthur-Bates Communicative Development Inventory (expressive language); Behavior Rating Inventory of Executive Function (BRIEF-P); Child Behavior Checklist 1.5-5 ADHD, PDD, attention; and reported diagnoses. Analyses were intent-to-treat. Mixed models compared treatment groups, controlling for clustering and baseline scores whenever possible. Sub-group effects for language by birthweight and for executive function by income were explored because adverse effects were observed in the original trial.

Results: Parents of 261/328 (80%) eligible children participated. No benefits of supplementation were observed at age 2 (see Table). The DHA+AA group had lower standard cognitive scores than the placebo group, but this was not statistically significant (median=107 vs 114, P=0.28). The DHA+AA group was twice as likely to have a current developmental/behavioral diagnosis (OR=2.02, 95% CI: 1.01, 4.05). In exploratory sub-group analyses, children with birthweight P=0.03, effect size=-0.44). Effects observed in the original trial on executive function by income were no longer present.

Conclusion: Long-term, robust assessment is needed to clarify the long-term effects of DHA+AA supplementation in toddlerhood for children born preterm.

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Gastroenterology & Nutrition

A Case of CLD Diagnosed In a Newborn After Surgical Treatment of Ileus

Jihye Kim ¹ , PilSang Jang ¹

¹ Department of Pediatrics, College of Medicine, The Catholic University of Korea, South Korea

Background: Congenital chloride diarrhea (CLD) is a rare autosomal recessive genetic disorder. The main symptom is osmotic diarrhea with high chloride content due to the mutations in solute carrier family 26 member 3 (SLC26A3) gene encoding the intestinal Cl^-/HCO₃^- exchanger. In Korea and other East Asian countries, only a couple of cases have been reported.

Case report: The patient was born at 34 weeks and 5 days of gestation and had a birthweight of 3.13 kg. He had a history of polyhydramnios and a noticeable dilated fetal bowel loops before birth. On day 1 of birth, the patient exhibited severe abdominal distension, vomiting, and biliary reflux. Because of suspected ileus, surgery was performed, and the surgical findings were ileus caused by Meckel`s diverticulum and consequent band formation. Wedge resection (diverticulum resection) of the ileum was performed, and the patient was discharged from the NICU with reduced symptoms. After discharge, however, he was hospitalized several times with intermittent postoperative abdominal distension and metabolite imbalances.

The patient, a 5-month-old male infant, was admitted with repeated vomiting and abdominal distension, accompanied with watery stool confused with urine. We suspected CLD and measured chloride level in stool. Watery stool was collected using a rectal tube, and the chloride level was 137 mEq/L, confirming excessive chloride in watery stool. On genetic testing, we confirmed SLC26A3 gene mutation, and CLD was diagnosed.

Discussion: This case report describes an infant who initially underwent surgical treatment for ileus induced by Meckel`s diverticulum and congenital band. Even after surgery, the patient continued to exhibit abdominal distension. The initial suspicion should be made on prenatal ultrasound, and patients with severe abdominal distension and chronic diarrhea should be tested for the possibility of CLD. Stool examination confirmed high-chloride content diarrhea and genetic testing led to a final diagnosis of CLD.

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Gastroenterology & Nutrition

An Audit of Maintenance Intravenous Fluid Therapy in the Paediatric General Wards at Mater Dei Hospital, Malta

Thomas Calleja ¹ , Jamie Grech ¹ , Iryna Kononenko ¹ , Choon Yee Wang ¹ , Nikita Taliana ¹ , Raymond Parascandalo ¹

¹ Paediatrics and Child Health, Mater Dei Hospital, Malta

Intravenous fluids are one of the most frequently prescribed drugs in hospital, and yet the practice continues to fall short of National Institute for Health and Care Excellence (NICE) guidelines. Paediatric patients are particularly vulnerable to complications of intravenous fluid therapy. We compare current practice in intravenous fluid prescription in the paediatric general wards at Mater Dei Hospital, Malta, to the standard as stipulated by the NICE guidelines. We show how standards of intravenous fluid prescription and subsequent monitoring by staff falls shorts of NICE guidelines, and make recommendations for improvement. Interventions were made to make staff aware of ongoing issues. A second audit cycle is ongoing.

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Gastroenterology & Nutrition

Complaints, Endoscopic and Histopathological Findings in Patients with Helicobacter Pylori Infection and their Correlation with Each Other

Gunsel Kutluk ¹ , Esra Polat ¹ , Nermin Gunduz ²

¹ Pediatric Gastroenterology, University of Health Sciences, Istanbul Kanuni Sultan Suleyman Education and Research Hospital, Turkey

² Pathology, University of Health Sciences, Istanbul Kanuni Sultan Suleyman Education and Research Hospital, Turkey

Objectives and study:There is no significant clinical state indicating H.pylori infection in pediatric population.In this study, most common complaints,endoscopic and histopathological findings in children with H.pylori infection were evaluated and their correlation with each other was explicated.

Methods:Patients between 3-18 years,applied to the hospital with dyspepsia,epigastric pain,reflux symptoms,anemia and suspected gastrointestinal(GI)bleeding and underwent upper GI endoscopy were enrolled in this study.Gastric antrum and corpus biopsies were performed in all patients during endoscopy for rapid urease test and histopathological examination.Sydney classification was used to evaluate H.pylori intensity,activity of gastritis,chronic inflammation,atrophy and intestinal metaplasia.Demographical properties, complaints,endoscopic findings and Sydney scores of the patients were recorded.

Results: A total of 339 patients(183 female)were enrolled in the study.The most common complaints were dyspepsia and epigastric pain.90% of the patients had antral pathology, 78% of which was nodularity.Relation between dyspepsia, epigastric pain and antral nodularity is found to be statistically significant.In histopathological examination, intensity score of H.pylori is found to be increasing with the age.Rapid urease test was positive in 90.2% patients.Relation between rapid urease test and the intensity of H.pylori was statically significant.Relations between detected morphological changes in the antrum and the intensity of H.pylori,activity and chronic inflammation scores were statistically significant.Intestinal metaplasia(IM)and gastric atrophy(GA)was present in 7.1% and 6.2% of the patients respectively.No relation was found between IM or GA with the intensity of H.pylori, endoscopic findings and complaints.

Conclusion: The most common complaints of children with H.pylori infection were epigastric pain and dyspepsia.There were significant relations with this complaints,antral morphological changes and with the intensity of H.pylori,and the intensity of H.pylori,increases with age.Since these complaints were related with antral morphological changes and these changes were related with activity of gastritis,chronic inflammation and increased intensity of H.pylori, early eradication can be recommended in these children in order to prevent from long term complications.

1050

Gastroenterology & Nutrition

Functional Features of the Duodenum in Children with the Syndrome of Bacterial Overgrowth in the Small Intestine

V.P. Novikova ¹ , M.M. Gurova ¹ , A.P. Listopadova ¹ , O.M. Tsekh ¹ , E.Yu. Kalinina ² , E.A. Orishak ³ , A.N. Petrovsky ²

¹ Laboratory of Medical and Social Problems in Pediatrics SIC, St. Petersburg State Pediatric Medical University, Russia

² Department of Pathological Anatomy, North-Western State Medical University named after I.I. Mechnikov, Russia

³ Department of Medical Microbiology, North-Western State Medical University named after I.I. Mechnikov, Russia

Objective: To study the relationship between the functional state of the duodenum and its microbiocenosis in patients with duodenitis associated with Helicobacter pylori (HP) infection.

Materials and Methods: In our study, we included 60 adolescents aged 14 to 18 years with morphologically proven duodenitis. We detected HP by PCR in feces. Bacteriological examination of the duodenal contents extracted during endoscopy via the endoscope biopsy channel was performed. The activity of enzymes in duodenal biopsy specimens was determined according to the method of A.M. Ugolev and N.M. Timofeev.

Results: It was revealed that helicobacteriosis without syndrome of intestinal bacterial overgrowth (SIBO) did not affect the activity of the brush border enzymes. In case of combination of helicobacteriosis and SIBО we found it negative correlation with the activity of lactase (r = -0.489, p 0.05) and aminopeptidase M (r = -0.487, p 0.05). Combination of helicobacteriosis and giardiasis had a negative correlation with the activity of lactase (r = -0.33, p 0.05) and glycyl-L-leucine dippeptidase (r = -0.324, p 0.05), whereas the combination of helicobacteriosis with SIBО and giardiasis reduced the activity of all three enzymes: lactase (r = -0.432, p 0.05), aminopeptidase M (r = -0.461, p 0.05) and glycyl-L-leucine dippeptidase (r = -0.374, p 0.05). We have identified microorganisms that have a negative effect on the lactase activity: Actinomyces (r = -0.318, p 0.05), Staphylococcus species (r =-0.416, p 0.05), Pseudomonas aeriginosa (r =-0.435, p 0.05) Peptococcus species (r =-0.455, p 0.05), Candida albicans (r =-0.346, p 0.05), Enterococcus faecalis (r = -0.426, p 0.05). We found a positive correlation between the presence of lactobacilli in the small intestine and the activity of aminopeptidase M (r = 0.341, p 0.05).

Conclusions: The microbiocenosis of the duodenum in adolescents with HP-associated duodenitis affects the activity of the brush border enzymes.

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Gastroenterology & Nutrition

Battery Ingestion in Children: A Pediatric Tertiary Care Centre Experience

Cristina Lorenzo ¹ , Sara Azevedo ¹ , Ana Paula Mourato ¹ , Helena Loreto ¹ , Miroslava Gonçalves ² , Ana Isabel Lopes ¹

¹ Pediatric Gastroenterology Unit, Department of Pediatrics, Hospital de Santa Maria- Centro Hospitalar Universitário de Lisboa Norte, EPE, Portugal

² Pediatric Surgery Unit, Department of Pediatrics, Hospital de Santa Maria- Centro Hospitalar Universitário de Lisboa Norte, EPE, Portugal

Background: Battery ingestion is a frequent problem in pediatric population and can lead to potentially serious complications, especially when located in the esophagus.

Objective: Describe battery ingestion cases requiring endoscopic removal and their complications.

Methods: Retrospective descriptive study of admissions for battery ingestion requiring endoscopic removal in a pediatric emergency department of a tertiary hospital (January 2009/December 2018).

Results: 29 cases, with an increasing number in the last four years (2018: 9 cases). Median age 26 months (8 months-9 years), 52% male. Witnessed ingestion in 24 (83%) patients; 16 (41%) patients were symptomatic, vomiting (21%) was the most frequent symptom. On the X-ray, 8/29 patients (28%) had an esophageal battery (EB) and 21/29 (72%) had a gastric battery (GB). The upper endoscopy revealed the battery (100% button lithium batteries) located in the esophagus (8), stomach (15), duodenum (1) or was not detected (5); median time until removal of EB was 21 hours (6h-21 days). All patients with EB presented with lesions of different severity: erosion (8), ulceration (6), necrosis (5) and perforation (2); 3 patients presented more than one lesion. Of all the cases of GB only a minor erosion was described. There were no cases of mortality reported.

Patients with EB had a more complicated clinical evolution, with prolonged hospital stay than GB [median 26 days (6days-80days) vs 14 hours (8hours-6days)] and prolonged follow-up [median 3 months (1month-8year)] vs 1.25 month (1month-1.5month)]; 2/8 (25%) had severe stricture after ingestion and are currently in endoscopic dilation program; one of them is being fed by gastrostomy.

Discussion: There has been an increasing number of battery ingestion in recent years. Esophageal batteries remain responsible for persistent severe lesions, mostly in patients with late removal. This study emphasizes the importance of primary prevention.

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Gastroenterology & Nutrition

Meta-Analysis of 196 Articles: Fatty Acid Supply of Expecting Women during Pregnancy and at Delivery in Erythrocyte Membrane Phospholipids

Tamas Marosvolgyi ¹ , Aniko Konig-Peter ¹ , Tamas Decsi ² , Eva Szabo ³

¹ Institute of Bioanalysis, University of Pecs, Hungary

² Department of Paediatrics, University of Pecs, Hungary

³ Department of Biochemistry and Medical Chemistry, University of Pecs, Hungary

Background: Long-chain polyunsaturated fatty acids are important for the maturation of the developing nervous system. As fetal fatty acids originate from maternal stores, the fetal as well as the neonatal supply are determined by the maternal fatty acids.

Objective: To systematically review available data on fatty acid composition of plasma and erythrocyte membrane lipids in expecting women during pregnancy and at delivery.

Methods: We performed electronic literature search in July 2017 on Cochrane Library, Embase, and Ovid Medline with a search strategy including the following search expressions: (pregnant* OR gestation* OR deliver*) AND (arachidonic OR docosahexaenoic) NOT animal. We analysed data from clinical trials investigating fatty acid composition of plasma or erythrocyte membrane lipids in healthy expecting women without any fatty acid supplementation during pregnancy. Statistical analysis of fatty acid data was performed by Comprehensive Meta-Analysis v3.0 Software.

Results: After excluding duplicates there were 3351 potentially relevant articles. We excluded 3155 articles because they didn’t meet our inclusion criteria. 196 relevant articles remained that published fatty acid data of plasma or erythrocyte membrane lipids, from them 19 articles published data of erythrocyte membrane phospholipids. Only 7-9 studies investigated the fatty acid composition of erythrocyte membrane phospholipids during pregnancy, while at delivery there were 13 articles publishing fatty acid data. Values of arachidonic acid remained quite stable in the course of pregnancy as well as at delivery. In contrast, values of docosahexaenoic acid were significantly lower during the 1^st trimester than in later timepoints and then it remained quite stable

(Table)

Conclusions: 1. Our results indicate that on a population level both docosahexaenoic acid and arachidonic acid status remain quite stable during pregnancy. 2. Our present data can serve as reference values for future fatty acid supplementation studies in expecting women.

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Gastroenterology & Nutrition

Food Protein-Induced Enterocolitis Syndrome in a Breastfed Infant

Miguel Martins ¹ , Isabel Azevedo ¹ , Nuno Lourenço ¹ , Sofia Ferreira ¹ , Susana Almeida ² , Ricardo Ferreira ² , Carlos Rodrigues ¹

¹ Pediatrics Department, Centro Hospitalar Universitário Cova da Beira, Portugal

² Pediatric Gastroenterology Unit, Hospital Pediátrico de Coimbra, Centro Hospitalar Universitário de Coimbra, Portugal

Background: Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated food allergy that occurs following the ingestion of a triggering food. Symptoms include incoercible vomiting and diarrhea 1-4 hours post exposure, and in severe cases dehydration and shock. This rare condition usually presents in infants younger than 9 months, with cow’s milk protein (CMP) as the main trigger. Breastfed infants seem to be protected against CMP-FPIES, although a few cases have been reported with exclusive breastfeeding. Diagnosis is clinical relying on a high suspicion.

Clinical Case: A 2-month-old girl exclusively breastfed (except for single bottle of regular formula at day-1) was admitted with intermittent bloody and mucous diarrhea, significant weight loss and dehydration. Laboratory findings showed hypoalbuminemia, thrombocytosis, mild hyponatremia and metabolic acidosis. Breastmilk was replaced by a semi-elemental formula (SEF), with clinical improvement. Once breastmilk was reintroduced the symptoms recurred. The infant was kept on a CMP free diet with regular growth and no gastrointestinal symptoms. A first oral food challenge (OFC) to CMP was performed around 9 months and was positive. Last OFC at 36 months remained positive. Diagnosis of FPIES caused by CMP passed through breastmilk was established. Currently, at age 4, she continues to thrive well under CMP restriction.

Discussion: Assessment of FPIES is difficult since it’s an exclusion diagnosis. Our case enlightens the importance of considering this condition whenever typical symptoms ensue, even in exclusively breastfed infants. Misdiagnosis or delayed diagnosis are common and may lead to a critical life-threatening situation.

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Gastroenterology & Nutrition

BL Whaaat? Baby Led Weaning: The Literature and How Families do it

Rita Matos Parreira ¹ , Gonçalo Vale ¹ , Mariana Simões ¹ , Maria São Pedro ¹ , Isabel Bretes ¹ , Cristina Didelet ¹

¹ Pediatrics Department, Centro Hospitalar Barreiro-Montijo, Portugal

Background: For the last 10 years Baby Led Weaning (BLW) has gained popularity as an approach to complementary feeding. It’s a hot topic on social media, blogs, parenthood books and forums. Parents keep asking about it in routine appointments.

BLW is a feeding approach that advocates bypassing conventional weaning practices, based in spoon-feeding of puréed food, to presenting whole foods to infants over 6 months.

Objective: There are few studies or long-term outcomes, but the number is increasing corroborating the method’s popularity and value.

The main purpose of this presentation is to clarify the method, either in its strict form or mixed with the conventional approach, showing its pros and cons, with the ultimate goal of making it easier for the pediatricians to answer parents` questions.

Methods: Summary of BLW’s theoretical basis, literature review and examples of its applicability in Portuguese families, based on clinical practice.

Conclusion: The authors pretend to present a plain and practical perspective, based on what is published in literature and share the heterogeneous experience within Portuguese families.

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Gastroenterology & Nutrition

A Rare Case of Abdominal Distension

Joana Monteiro ¹ , Joana Monteiro ¹ , Tânia Mendo ¹ , Gabriela Reis ¹ , Maria Carlos ¹ , Sónia Fernandes ¹ , Isabel Lança ¹ , Filipa Santos ² , Aniceta Cavaco ¹

¹ Pediatric, Hospital José Joaquim Fernandes, Portugal ² Pediatric Gastroenterology, Dona Estefânia Hospital, Portugal

Background: There are several causes of abdominal distension, elongation and dilatation of the colon (dolichomegacolon) is a rare diagnosis in pediatric age. It may be congenital or acquired; chronic or acute and may lead to intestinal oclusion and perforation of the intestinal wall, resulting in sepsis and risk of death.

Case Report: A 7 month old male child was referred to the paediatric department for abdominal distension, poor weight progression and abundant stools. First child of young non-consanguineous parents (father with esofagogastric reflux) with no hereditary diseases. His birth and psicomotor development were normal. Under exclusive breast milk up to 4.5 months and because of poor weight (15th centil up to 4 months and 3-15th centil since then) starts infant formula. Introduction of gluten at 6 months. Hospitalization for acute gastroenteritis at 3 months. On examination he had a distended abdomen, soft on palpation with tympanism. Blood investigations showed anemia (Hb 10,8g/dL), ferritin 27ng/mL and PCR 0,2mg/dL. Hepatic and renal function, total protein and ionogram had normal values, as well as his immunoglobulin concentrations. Celiac disease, cow`s-milk protein allergy, cystic fibrosis and giardisis were excluded. Upper endoscopy with biopsy (mild degree of esophagitis and gastritis), high resolution manometry, rectoanal inhibitory reflex and sensitivity to rectal distention were normal. Lower gastrointestinal series showed a dolichomegacolon. He was treated with proton-pump inhibitor, laxatives, probiotics, high-fiber, lactose-free diet.

Conclusion: The dolichomegacolon justifies multiple recurrencies to the emergency room presented with constipation or intestinal suboclusion and has a difficult treatment.

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Gastroenterology & Nutrition

Coeliac Disease (CD) and Gastropathy in Paediatric Practice

David Croaker ¹ , Himasha Nanayakkara ²

¹ Department of Paediatric Surgery, The Canberra Hospital, Australia

² Medical School, University of Notre Dame, Sydney, Australia

Background: Reactive gastropathy is a defensive change in the stomach. In adults, it is caused inter alia by NSAID use and bile reflux. Histological features of reactive gastropathy include foveolar hyperplasia, mucosal congestion and hyperplastic smooth muscle . Reactive gastropathy is associated with various inflammatory conditions.

Objective: The authors sought to characterise the association between reactive gastropathy and coeliac disease in children.

Methods: A retrospective chart review on paediatric gastroscopies conducted between 2003-2018 was used. The data was stored in Excel. All patients with coeliac disease or any gastropathy were identified by histopathology. The presence of gastropathy was noted in those with proven coeliac disease. The presence of bile in gastric contents was also recorded as this could act as a potential confounder.

Results:1779 gastroscopes were reviewed. 1279 results were available for the histopathological diagnosis of the stomach. 437 had reactive gastropathy, a prevalence of 24%. Of those with reactive gastropathy, 143 had confirmed CD. 842 did not have gastropathy. Of this group, 149 had confirmed coeliac disease. Fisher’s exact test showed a significant difference between the results (p0.05).

Of the 143 confirmed CD diagnoses within the gastropathy group, there were 81 cases of gastric contents recorded. In 231 cases recorded overall, there was no association between the presence of bile in the stomach and gastropathy. (NS using Fisher’s exact test.) Overall 195 of 294 with CD recorded an abnormal gastric biopsy, 3 of them lymphocytic gastritis. 12 of 294 (4%) had eosinophilic oesophagitis (EoE).

Discussion: Gastropathy of various types is common at endoscopic pathology. Reactive gastropathy, distinct from lymphocytic gastritis, is common in CD in paediatrics, and EoE may also be more common than observed background rates. Bile stained gastric contents did not explain the gastropathy, and reactive gastropathy is not confined to CD patients.

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Gastroenterology & Nutrition

Is This Caroli Disease?

Irena Nikolova ^1,2 , Marija Dimitrovska-Ivanova ^1,2 , Luiza Doneva ¹ , Marija Karagozova ¹

¹ Department of Pediatrics, Clinical hospital Shtip, Macedonia

² Department of Pediatrics, University Goce Delcev Shtip, Macedonia

Introduction: Caroli disease is rare congenital disorder of the intrahepatic bile ducts. It is characterized by dilatation of intrahepatic biliary tree and with renal cysts. Caroli disease is inherited autosomal dominant.

Aim: To present a case of seven months old infant with icterus of the skin and sclera.

Material and methods: Seven month old infant with gray-yellow skin color and fatigue for a month. Positive family history of policystic renal disease and carcionama of the liver. At admittion afebrile, pale, yellow sclera. Abdomen above torax. On palpation with hepatosplenomegaly. From examinations: WBC -17,07 CRP= 12.9mg/l. Tot.Bill= 141,5umol/l, Dir. Bill= 93.3umol/l, AST= 142U/L, ALT= 143U/L, ALKP= 790 U/L, LDH= 415U/L, CK=330U/L, CA-19-9 =399,7 U/ml. Abdominal ultrasound showed more cystic formations in the liver that are connected in parts and a larger cyst in the hepatoiduodenal ligament and the liver hilus. The finding goes in addition to Caroli disease. CT scan of abdomen and MRI cholangiopancreatography showed hepatomegaly, cystic and sacular dilated intra and extrahepatic bile pathways. Both kidneys are without signs of obstruction, but with cortical cysts, right in the upper parts and left in the interpolar region with diameter 15 mm. Mild intestinal distension.

CT scan of abdomen

MRI cholangiopancreatography

Results: Child is outpatient followed by a Pediatric gastroenterohepatologyst and Pediatric surgeon. Child recives Ursodeoxycholic acid and the last results with withdrawl of liver enzymes and bilirubin. HLA typisation is planed for liver transplatation in the future.

Conclusion: Although this disease is rare, hyperbilirubinaemia of direct type and a positive familial history of polycystic kidney should lead us to think of Caroli disease.

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Gastroenterology & Nutrition

Late Diagnosis of Right Congenital Diaphragmatic Hernia: Why Didn`t I Think About It Sooner?

Andreea Stefania Nedelcu ¹ , Raluca Maria Vlad ^1,2 , Daniela Pacurar ^1,2

¹ Pediatrics, “Grigore Alexandrescu” Emergency Hospital for Children, Bucharest, Romania, Romania

² Pediatrics, “Carol Davila” University of Medicine and Pharmacy, Romania

Background: Diaphragmatic hernia is not a common disease. The severity of symptoms may vary depending on the size of defect and the organs involved.

Objectives: The authors aim to raise the awareness regarding this congenital condition as it may not be clinically obvious for a long time.

Methods: We present two cases from the Pediatrics Department of “Grigore Alexandrescu” Hospital, patients with different types of presentation incidentally diagnosed with congenital diaphragmatic hernia after the age of three.

Results: Case 1: 8 year old boy presented with jaundice. The blood tests revealed elevated levels of total bilirubin, normal liver enzymes. The abdominal ultrasound described a liver mass, possibly a vascular malformation. The patient underwent MRI that excluded this supposition. A thoracic x-ray revealed a right, paracardiac, dome-shaped opacity. The diagnosis was right diaphragmatic hernia with liver thoracic dislocation. Case 2: 3 years 6 months old girl is admitted for persistent cough. An antenatal diagnosis of agenesis of the aortic arch had been set. She was born premature, required intensive care during the neonatal period, one diagnosis being right pulmonary hemorrhage. Postnatal ultrasound examination diagnosed venous duct anomaly (hepatic veins draining in the right atrium). She had a good clinical course with normal growth, cardiac and liver functions. The clinical examination was unremarkable. A thoracic x-ray described homogeneous, well-defined opacity in the in the right lower pulmonary lobe. She underwent antibioticotherapy for pneumonia (7 days) with no change of the thoracic image. Abdominal ultrasound confirmed complex malformation of the venous duct (azygos continuation of the inferior vena cava). Thoracic computed tomography demonstrated Morgagni’s hernia with liver thoracic dislocation.

Conclusion: Diaphragmatic hernia is a challenging diagnosis to be made. Although a congenital condition, it may be asymptomatic for a long time and have many different clinical “faces” at presentation.

474

Gastroenterology & Nutrition

Preventive Treatment of Various Complications in the Management of Short Bowel Syndrome and Gastrointestinal Neuromuscular Diseases

Dionisiy Petrov², Iuliia Aver'ianova¹, Sergey Makarov¹, Elena Zhelezoglo², Anastasia Ermolaeva

¹ Department of Pediatric Surgery, Russian Children's Clinical Hospital of Ministry of Health of Russia, Russia

² Department of Pediatric Surgery, Pirogov Russian National Research Medical University, Russia

Aim: To demonstrate the approach for prediction and management of different complications of chronic intestinal failure (IF) caused by short bowel syndrome (SBS) and gastrointestinal neuromuscular diseases (GINMD).

Methods: 58 patients with SBS and 44 with GINMDs aged 1 month to 17 years have been treated in the Department of Pediatric Surgery at Russian Children`s Clinical Hospital. We provide a full range of non-transplant options for IF management: home parenteral nutrition (HPN) program, autologous intestinal reconstructions (AIR), absorption-enhancing drug therapy etc. The most common complications included: catheter-associated bloodstream infections (CABSI) (33,3 %), invasive Candidemia (20,4 %), Intestinal Failure Associated Liver Disease (IFALD) (4,9 %), metabolic bone disease (24,5 %), small intestinal bacterial overgrowth (SIBO) and translocation (11,8 %), recurrent D-lactic acidosis (5,9 %).

Multiple measures are being made to predict and prevent these complications. Thorough training of parents on central line care and PN administration, routine use of antibacterial locks have helped to minimize CABSI. The use of new-generation lipid emulsions and choleretic agents allowed to reduce the number of IFALD. For metabolic bone disease the total serum 25(OH)D level and the level of trace elements are being monitored; parenteral Vitamin D and its active metabolites, biphosphonates and calcium/phosphorus oral supplements are prescribed in accordance with clinical presentation. A combination of pre- and probiotics with rifaximin is used to manage SIBO and recurrent D-lactic acidosis.

Results: SBS patients who underwent AIR revealed a statistically significant dynamics of a decrease in the number of catheter-associated complications, IFALD, invasive Candidemia. In patients with severe GINMDs with recurrent intestinal obstructions we put G-tube and create a T-shaped enteroenteroanastomosis which allows to improve gas evacuation and reduce SIBO.

Conclusions: patients with SBS and GINMDs require a multidisciplinary approach. Prediction and prevention of complications improve the quality of life and shows a great economic value.

950

Gastroenterology & Nutrition

Knowledge, Attitude, and Intention of Breastfeeding among Male and Female University Students

Hadia Radwan, Mona Hashim, Sara Mustafa, Sarah Idris, Layan Al Shalabi, Noor Madan Madan

¹ Clinical Nutrition and Dietetics, University of Sharjah, United Arab Emirates

Background: Breastfeeding is an important public health strategy for improving short-term and long-term health consequences of the mothers and their infants. Paternal breastfeeding knowledge and intention may influence the maternal choice of breastfeeding.

Objective: The purpose of this study was to examine university undergraduate females’ and males’ knowledge, attitudes and intention toward breastfeeding.

Methods: A cross-sectional study was conducted among 492 university students included 300 female and 192 male. A questionnaire with sociodemographic information and validated scales to explore: Exposure to breastfeeding, knowledge about Breastfeeding, Iowa Infant Feeding Attitude Scale, and Infant Feeding Intentions was administered online to the university students.

Results: The participants’ exposure to breastfeeding was considerably high(89.8%). There was a highly significant positive correlation between sex and the knowledge and attitude scores (r=0.22, r=0.26, P=0.001) where females had more knowledge and positive attitude towards breastfeeding compared to males. Whereas males scored higher on intention scale (P0.001). Furthermore, there was a highly significant positive correlation between knowledge and attitude among the participants (r=0.46, P0.001 ). More than half (57.5% ) of the students agreed that breast milk is the ideal food for babies and that breastfeeding is healthier than formula feeding and would increase mother-infant bonding. However, the majority of the participants reported low intention to breastfeed and that they will only formula feed their babies (75.8%). There was a highly significant inverse association between knowledge scores and intention (r =-0.26, P0.001).

Conclusions: Overall, the findings from this study revealed that although both males and female university students had good knowledge and attitude about breastfeeding, their intention to breastfeed was low. This is alarming and necessitates that educational programs should promote breastfeeding in schools and universities in order to increase awareness and knowledge about the short term and long term benefits of breastfeeding

586

Gastroenterology & Nutrition

A Girl with Jaundice in The Emergency Department

Gabriela Reis ¹ , Tânia Mendo ¹ , Joana Monteiro ¹ , Maria Carlos ¹ , Sónia Fernandes ¹ , Ana Fernandes ² , Ana Isabel Lopes ²

¹ Serviço de Pediatria, Unidade Local de Saúde do Baixo Alentejo, Portugal

² Unidade de Gastrenterologia Pediátrica, Departamento de Pediatria, Hospital de Santa Maria - Centro Hospitalar e Universitário de Lisboa Norte, EPE, Portugal

Background: Autoimmune hepatitis (AIH) is an inflammatory liver disease, characterized by circulating autoantibodies, increased IgG levels and liver biopsy with parenchymal inflammation. In the absence of early treatment, it may progress to cirrhosis and liver failure. The acute clinical presentation with asthenia, abdominal pain, anorexia, vomiting, jaundice and headache, occurs in 50% of patients.

Case Report: Previously healthy 11-year-old girl, observed in the emergency department for fever (temperature 38.6°C, every 6 hours), cough, rhinorrhea, frontal headache, weariness and anorexia for 8 days. No relevant personal, family or medical history. No history of recent infections. She had subicteric sclerotics, with no other relevant findings. Blood tests showed mild elevation of inflamatory parameters (WBC’S 13.160 U/L; N 75.3%; CRP 1.1 mg/dL), cytolysis (AST 433 UI, ALT 708 UI), without cholestasis (FA 264 UI, GGT 82 U/L, Total Brb 1.97mg/dL , Direct brb 0.95 mg/dL), LDH 288 U/L and normal tests for clottig time. Chest x-ray was normal. Abdominal ultrasound showed hepatosplenomegaly. The diagnosis of viral hepatitis was presumed. Due to the absence of severity criteria, she was discharged, and reassessed 24 hours later. From the additional investigation performed, the virulogical study was negative, and Wilson’s disease, alpha-1-antitrypsin deficit and hemochromatoses were excluded. Autoimmunity studies showed positive ANA, doubious anti-Dsdna antibodies and positive anti-LKM1 (LC1) antibodies. The patient was transferred to a tertiary Hospital, where underwent liver biopsy, whose result was compatible with autoimmune hepatitis. She started therapy with prednisolone and hyposaline diet, followed by azathioprine, with clinical and analytic improvement.

Conclusion: Although a large group of patients present with thypical symptoms, sometimes the ones that bring them to the ER are not the most common. Therefore, the authors intend to demonstrate the importance of a thorough physical examination and high degree of clinical suspicion, for a good diagnostic orientation.

206

Gastroenterology & Nutrition

Carotenoids Intake and Skin Carotenoids Level in Asthmatic Thai Children

Lakkana Rerksuppaphol ¹

¹ Department of Preventive Medicine, Srinakharinwirot University, Thailand

Background: The prevalence of asthma is a phenomenon dramatically increasing worldwide. Several antioxidant nutrients including carotenoids have been described to inversely correlate with asthma. The majority of studies quantify carotenoids in relation to the amount of food intake rather than direct measurement of tissue carotenoids.

Objective: The study aims to assess carotenoids intake and skin carotenoids levels in asthmatic children compared to control group.

Methods: A cross-sectional school-based study was conducted in 423 schoolchildren, aged 3.5-17.8 years, in one school at Ongkaluck district, a rural region in central Thailand. Asthmatic children were diagnosed accordingly to Health Interview for Asthma Control questionnaire. Skin carotenoids level was measured with Raman spectroscopy. Demographic data were obtained by directly interviewing children and their parents, whereas anthropometric parameters were measured by trained staff. The intakes of carotenoids were evaluated by a food frequency questionnaire. Pearson chi-squared test and Student’s t-test were used to compare categorical and continuous variables between asthmatic and non-asthmatic children, respectively. Correlations between skin carotenoids level and carotenoids intake were measured by Pearson’s correlation test.

Results: Seventy three children (17.3%) had diagnosed asthma. There were no significant differences in term of anthropometric parameters, dietary intakes of α-carotene (904 vs 1,159 mcg/week; P=0.65), β-carotene (5,232 vs 5,189 mcg/week; P=0.60), β-cryptoxanthin (1,028 vs 1,045 mcg/week; P=0.39), lycopene (4,417 vs 4,951 mcg/week; P=0.54), lutein and zeaxanthin (6,077 vs 6,098 mcg/week; P=0.54) between asthmatic and non-asthmatic children. The mean skin carotenoids level was 12,295.6 RU in asthmatic children and 12,529.6 RU in control group (P=0.95). Skin carotenoids level significantly positive correlated with all carotenoids intake in a mild to moderate degrees (r= 0.20-0.42, P0.05).

Conclusions: Carotenoids intakes and skin carotenoids levels were not associated with of the risk of asthma in Thai children. Skin carotenoids correlated with all carotenoids intake in a mild to moderate degrees. Raman spectroscopy was a useful tool to determine skin carotenoids level.

204

Gastroenterology & Nutrition

Zinc Supplementation in Thai Children with Dengue Fever

Sanguansak Rerksuppaphol ¹

¹ Department of Pediatrics, Srinakharinwirot University, Thailand

Background: Zinc deficiency is common in developing countries and increases the risk for several infectious diseases. Low serum zinc levels have been reported in children with dengue virus infection (DVI).

Objective: To assess the effects of zinc supplementation on DVI outcomes.

Methods: A double blinded, randomized trial was conducted in children with dengue fever (DF)/ dengue haemorrhagic fever (DHF). Bis-glycinate zinc or placebo was orally administered 3 times a day for 5 days or until defervescence. Primary outcome was to evaluate the DVI defervescence phase; secondary outcome was to assess hospitalization length, presences of severe DVI and zinc deficiency.

Results: Fifteen children with DHF and 35 with DF were randomized 1:1. Overall prevalence of zinc deficiency was 46%. Serum zinc levels increased from baseline to the end of study: the mean gain was 26.4 mg/dL (95%CI: 13.6 to 39.1 mg/dL) in the supplementation group and 14.4 mg/dL (95%CI: 7.4 to 21.3 mg/dL) in placebo group. Mean time of defervescence was not significant different between groups (29.2±24.0 and 38.1±31.5 hours; P-value=0.270). Hospital staying was shorter for children with zinc supplementation than for those with placebo (mean difference 22.2 hours, 95%CI: 5.5 to 38.5 hours; P-value= 0.010). No signs of severe DVI were observed in both groups. Zinc supplementation was well tolerated.

Conclusion: Overcoming zinc deficiency among Thai children may reduce DF duration and limit the hospitalization, in addition to other advantages that normal serum zinc levels have on overall children health.

514

Gastroenterology & Nutrition

Dietary Habits and Body Esteem in Adolescents Survey

Ivanna Romankevych ¹ , Marina Mamenko ¹ , Olexiy Rykov ² , Stanislava Gaponova ¹ , Yuliia Bondarets ¹ , Hanna Drokh ¹

¹ Shupyk National Medical Academy of Postgraduate Education, Ukraine ² Dobrobut, Ukraine

Background: There are a lot of studies dedicated to anorexia nervosa in teens, but its general frequency isn’t so high. Still, adolescents are risk group for development of imbalanced diet as well as body dysmorphic disorders and low body-esteem. However, nutritional pattern and body-esteem in adolescents aren’t so well established.

The Aim: to evaluate dietary habits and body self-esteem in adolescents.

Material and Methods: 36 own design questionnaires, filled in by adolescents by their own. The questioner includes 3 blocks of questions: own evaluation of body composition and weight, dietary habits and adherence to a specific diet.

Results: After analyzing of data we received following results from 53%(19) boys and 47%(17) girls with mean age 14.75±0.46 years. There were 10.8%(3) adolescents with underweight, 7.2%(2) with overweight and 82%(31) with normal weight. However, all children with underweight considered their weight as normal, 25%(9) teens with normal weight count their weight as an overweight, but not as obesity. The 83%(30) of children evaluated body weight of parents as normal. Parents prepared food to all respondents. The 58%(21) teens consumed food 3-4 times, 25%(9) 2 times and 8%(3) 1 time daily. Consuming food without hunger was noted by 58%(21) of adolescents and eating in front of a TV was found in 75%(27) cases. There were 66.7%(24) ordered fast food at least 1 time weekly, 92%(33) drank soda or sweet beverages daily. However, 92%(33) and 83%(30) of children ate fruits and vegetables respectively every day. There were 18(50%) teens, which follow hypocaloric diet on purpose of weight reduction. We did not have respondents practiced vegetarian or vegan, or other specific diet.

Conclusions: survey showed features of misinterpretation of own weight and body composure, simultaneously with suboptimal dietary habits and adherence to hypocaloric diet in adolescents.

191

Gastroenterology & Nutrition

Utility of Probiotics in Gastroenteritis Treatment in the Paediatric Population

Abidur Rahman ¹ , Darolyn Tan ¹ , Nnedimma Ozoani ¹ , Sarah Koscic ¹ , Jia Jun Foo ¹ , Craig Duffy ¹ , Laken Boochoon ¹ , Allison Bell ¹ , Eleanor Molloy ¹

¹ Paediatrics, Trinity College Dublin, Ireland

Background: Acute gastroenteritis is the sudden onset of diarrhoea with passage of 3 or more loose stools in a 24 hour period, or the passage of 1 or more bloody stools in the presence or absence of abdominal pain, fever, nausea and vomiting. Acute gastroenteritis is self-limiting lasting 2 weeks or less, however, it is a major cause of morbidity and mortality worldwide. Current primary treatment is restoration of the acid-base balance, correction of electrolyte disturbances, and oral rehydration. The treatment helps to decrease morbidity and mortality, however, it seldom impacts the duration and symptoms of the infection. Probiotics may help reduce both the duration and severity of symptoms of gastroenteritis, however strain specific efficacy and inter-strain comparison has yet to be established.

Objective: This systematic review examines original research and the different strains of probiotics used to determine the efficacy of probiotics in treating acute gastroenteritis in the paediatric population.

Methods: An Embase search was carried out and the articles were screend by two reviewers. All reviewer conflicts were resolved by a third reviewer. Data was extracted from the included articles to determine correlation and effects of probiotics.

Results: 581 results obtained from the search. 11 studies were included for data extraction after applying the inclusion and exclusion criteria. Majority of the studies showed probiotics reduced duration of diarrhoea (8 of 11 studies), and duration of hospitalisation (6 of 11 studies). 2 papers reported adverse effects, such as fungaemia, in immunocompromised and ICU patients. The different strains of probiotics that were examined in the selected papers include Saccharomyces boulardii, Lactobacillus casei, and Lactobacillus acidophilus.

Conclusion; Probiotics reduced the duration of diarrhoea symptoms and hospitalization. Usage of probiotics was however accompanied by minimal side effects but not indicated in immunocompromised patients with gastroenteritis. Further research needs to be conducted to determine the strain specific efficacy and dosage requirements for treatment of gastroenteritis using large scale double blinded RCTs.

438

Gastroenterology & Nutrition

Paediatric Inflammatory Bowel Disease from 2008 to 2018: An Expending Problem in our Tertiary Unit

Cristina Becheanu ¹ , Gabriela Leşanu ¹ , Daniela Păcurar ¹ , Roxana Smǎdeanu ¹ , Evelina Cerbureanu ¹ , Iulia Ţincu ¹

¹ Gastroenterology Department, "Grigore Alexandrescu” Children Emergency Hospital, Romania

Background: The last two decades rapidly risen in the incidence and prevalence of childhood-onset inflammatory bowel disease (IBD) has demanded many research debates. Both developed and developing countries are facing the burden of early onset IBD, although previously it was considered to be rare in children.

Objectives: The aim of our study was to evaluate the characteristics of paediatric IBD patients recorded over a 10 year period, 2008-2018.

Methods: We identified children diagnosed with IBD using the database of „Grigore Alexandrescu” Clinical Emergency Hospital, a tertiary unit, from Bucharest, the capital of Romania.

Results: The total number of IBD paediatric cases investigated in our Department over a 10 years period is 74. In this interval, 62 patients (20 males/42 females= 0.477) were diagnosed with UC and 12 subjects had CD (7 males/ 5 females=1.4). The sex difference was not significant for neither CD nor UC (p0.05). The median age in years was 11.4±4.83 for IBD, 11.32±4.86 for UC, 11.81±4.87 for CD. The median age at diagnosis did not differ between CD and UC (p˂0.005), and evaluation regarding sex showed no significant differences between the age at onset in boys and girls (p˂0.005). The mean duration of time between age at first symptoms and diagnosis was 5.02±7.96, 4.96±8.33 and 5.33±6.00 months for IBD, UC and CD respectively. We report 17.6% patients with very early onset IBD, while the previous data from 2004 to 2015 showed 33% being diagnosed before the age of 6. In the last 3 years we identified 40 newly diagnosed IBD cases with mean age at diagnosis of 13.61±3.07 years.

Conclusion: Future studies should investigate the epigenetic factors, such as antibiotics use in the first years of life, feeding patterns and infections, among our paediatric IBD cohort, considering its increasing incidence and changing patterns over time.

443

Gastroenterology & Nutrition

Areas of Action against Later Childhood Obesity: Result of a 5 Years Prospective Study

Cristina Becheanu ¹ , Virginia Antoci ¹ , Andreea Gârbea ¹ , Iulia Ţincu ¹

¹ Gastroenterology Department, "Grigore Alexandrescu” Children Emergency Hospital, Romania

Background: Obesity is one of the greatest public health challenges in the world with childhood prevalence rates between 20–26% and numerous associated health risks.

Objective: The aim of this study was to analyze a spectrum of factors contributing early in life to overweight and obesity in a Romanian paediatric population, followed from 1 to 6 years.

Methods: Data on breastfeeding, early feeding patterns, individual anthropometric measurements and social characteristics were collected in a population-based prospective cohort study among 382 children. Of the original sample, only 113 (29.5%) subjects participated in the 5-year follow-up.

Results: The distribution according to BMI in terms of underweight, normal weight, overweight/obese children were 11.5% (13), 74.3% (84) and 14.2% (16) respectively, at 12 months old, while the proportions were 3.5% (4), 73.5% (83), and 23% (26) respectively, at the age of 6. The BMI evolution for 1 year underweight children was to normal weight in 76.92% cases and to overweight/obesity in 15.38% subjects, while 1 individual remained underweight. Sixty two per cent (62.5%) of the 1 year obese infants were still obese after 5 years. Univariate predictors of BMI in subjects aged 6 were identified as: 12 months BMI (r=0.36, p=000), duration of breastfeeding (r=-0.45, p=0.000), timing of complementary feeding (r=-0.54, p=0.000), amount of meat during weaning (r=0.63, p=0.000), while living area, family income, maternal age or education had no impact.

Conclusion: Timing of introduction of solid foods and breastfeeding duration were associated with child obesity at 6 years in our sample. Since reducing obesity is a difficult task once it appeared, preventing interventions should be implemented in the first years of life.

705

Gastroenterology & Nutrition

Recurrent Intestinal Invagination : Atypical Manifestation of Coeliac Disease

Ana Isabel Valdivielso Martnez ¹ , Silvia Gallego ¹ , Eva Pedrero Segura ¹

¹ Pediatrics, Primary care Attention, Spain

Background : Intestinal invagination usually presents accutely in infants under 2years after gastro-intestinal tract infection, when it occurs in older children or with chronic symptomatology , secondary causes must be ruled out such as polips, tumours or rarely, coeliac disease.

Objective: We present here an atypical case of recurrent invagination in a 9 years old girl wih malnutrition.

Methods: Clinical report of a case, clinical and laboratory data have been collected.

Results: 9 years old girl who lived in Morocco with her mother with low socioeconomic status. She used to have pica, failure to thrive and episodes of acute abdominal pain wih distension and vomits. She had no access to healthcare. She was transfered to emergency department in Spain hospital because of abdominal pain, vomiting and bloody diarrhea with ultrasound diagnosis of invagination. During surgery enlarged intestinal lymph nodes were seen and removed for biopsy and the invagination was reducted. Several malnutrition was noticed, with body mass index , Waterlow and Shukla index very low for his age; she presented with no eyelashes and eyebrows thinning (Picture1). In blood test, iron deficiency, low leves of vitamine A, E, B12, zinc and folic acid were measured, and very high levels of anti-transglutaminase antiboides IgA (128 U/ml). Duodenal biopsy was carried out and resulted in marked atrophy of villi and increased criptes (Marsh IIIb). After gluten-free diet the patient did not have invagination any more and gained weight.

Conclusion: Although coeliac disease is well-known and easily diagnosed in developed-countries, atypical and severe manifestations can occur when there is no access to medical services in non-developed countries.

1005

Gastroenterology & Nutrition

Severe Iron Deficiency Anemia - Consequences of Excessive Intake of Dairy Products in Children

Goncalo Vale ¹ , Rita Matos Parreira ¹ , Mariana Simões ¹ , Maria São Pedro ¹ , Joana Extreia ¹ , Sofia Castro ¹

¹ Pediatrics, Centro Hospitalar Barreiro Montijo, Portugal

Background: Iron deficiency can have several clinical consequences such as anemia or short and long-term developmental delay in children.

Iron deficiency anemia is multifactorial. It can occur due to problems of intestinal absorption, including excessive intake of dairy products, which interfere with the absorption of iron from other food.

Objective: Remind severe possible drawbacks of excess dairy food in pediatric age.

Methods: Report of a clinical case from a suburban hospital in Portugal.

Clinical Report: Male, two years and eight months old, ex premature with delayed psychomotor development. Gypsy ethnicity, consanguineous parents, family history of Favism. Artificial milk-based feeding since birth due to poor adaptation to breastfeeding and food diversification since eight months of age. Sent to an appointment due to failure to thrive, with all parameters inferior to percentile three according to WHO curves. Prostration and extremely pale mucosa were observed, being decided hospital admission for investigation. Analytical evaluation: Hemoglobin 4.8g/dl (normal 11,5-13,5g/dl), Hematocrit 20.3% (normal 34–40%), Iron 19ug/dl (normal 60-170 μg/dl), Ferritin1ng/mL (normal 15-300ng/ml); transferrin saturation 4% (normal 20-50%); Total capacity fixing iron 503ug/dl (normal 250-400 μg/dl). Peripheral blood with marked microcytosis and hypochromia, elliptocytes and dacryocytes. Abdominal ultrasound was unremarkable. Blood transfusion was performed and iron therapy was initiated with an improvement of all analytical parameters. During hospitalization, health professionals verified continuous intake of milk, instigated by parents, with detriment and refusal of other food by the child. Parents later admitted that infant feeding is milk-based at all meals.

Conclusion: Excessive milk ingestion is a common cause of iron deficiency in preschool age children. After six months of age, it is necessary to supply iron with complementary food and limit milk intake. Reinforcing this information and stimulating its practice to parents is extremely important for the prevention of anemia and its consequences in childhood.

594

Genetic & Metabolic

Contributing Factors to the Profound Incidence of Down Syndrome in the United Arab Emirates and Health Outcomes for Children with Down Syndrome in the UAE

Alya Ahmad ¹ , Alya Ahmad ¹

¹ Pediatric, University of California San Francisco at Fresno, USA

Purpose Down Syndrome rates are twice as high in the UAE. It is important to obtain the array of demographic, co-morbidities, and health outcomes of children with Down Syndrome (DS) in the UAE, Down Syndrome Gulf Registry

Methods: After IRB approval, a 3-year retrospective chart review of health data for 178 patients presenting to the DS Clinic were collected and analyzed using SPSS software. Systems-based medical co-morbidities associated with Down syndrome and 20 demographic, socio-economic, educational, and maternal age indicators were documented.

A Standardized template of their care plan was incorporated into an EHR system using 2011 AAP DS Health supervision guidelines in a UAE Down Syndrome Clinic. The children with DS were enrolled in the DS registry preventative health and complex care coordination was provided.

Results: DS in the UAE is starkly 2-2.5 times greater as global incidence of DS.

Conclusions: Children with Down syndrome living in the UAE share similar characteristics with other populations. However, there are some interesting differences in both demographics and co-morbidity patterns. High rates of parental consanguinity, no prenatal screening, and increasing maternal age maybe a factor in increased incidence. Genitourinary anomalies occur frequently, as do the other commonly described medical co-morbidities. Similar cardiac malformations occur, but AVC is not the most common. Higher co-morbidity rates and surgical are noted in the DS clinics of a tertiary hospitals. Select cases with severe compromise highlight the need to follow medical guidelines to optimize outcomes. Further development of the registry to improve outcomes in sleep apnea monitoring, obesity, and other long-term progressive conditions needs to occur

1055

Poster presentation

Effect of Pamidronate Therapy in Children with Osteogenesis Imperfecta

Naima Baddouh ¹ , L. Bourkhisi ¹ , S. Mghar ¹ , M. Lagrine ¹ , A. Ouayad ¹ , H. Nassih ¹ , A. Bourrahouat ¹ , I. Ait Sab ¹

¹ Pediatrics B Department, Mother and Child Hospital, Mohammed VI University Hospital Faculty of Medicine, Cadi Ayyad University, Morocco

Osteogenesis imperfecta (OI), an inherited connective tissue disorder of remarkable clinical variability, is caused by a quantitative or qualitative defect in collagen synthesis and is characterised by bone fragility. A multidisciplinary team approach is essential for diagnosis, and to tailor treatment needs to the severity of the disease and the age of the patient.

Objective: To analyze the clinical and radiological aspects of this condition and to evaluate the response to pamidronate therapy in children with moderate to severe osteogenesis imperfecta.

Methods: This work is a retrospective study with a prospective follow-up of 18 patients, collected at the Pediatrics B department, Mother and Child Hospital, Mohammed VI university hospital, Marrakech, Morocco, during a period spanning 4 ½ years, from January 2013 to June 2017.

Results: clinical presentation was characterized by multiple fractures and deformities in 89% with 11% of cases of antenatal diagnosis. Dentinogenesis imperfecta was present in 56% and blue or gray sclera was found in 94.5%.

All our patients were treated with pamidronate at age-appropriate doses. During the follow-up we found clinical and radiological improvement with a decrease in the fracture rate in all of our patients. Genetic counseling for parents was done.

Conclusion: This study suggests that treatment with pamidronate improves mobility in children with moderate to severe osteogenesis imperfecta and decreases the frequency of fractures.

292

Genetic & Metabolic

How do we Train Clinicians to Integrate Genomics into Paediatrics?

Bryony Coupe ^1,2 , Maurizio Brotto ^1,2 , Rhiannon Clegg ^1,3 , Helen Daniels ¹ , Jason Griffiths ^1,4 , Torsten Hildebrandt ^1,5 , Rachel Thomas ^1,6 , Ruth Young ^1,7 , Claire Morgan ¹

¹ Institute of Life Science, Swansea University Medical School, UK

² Singleton Hospital, Abertawe Bro Morgannwg University Health Board, UK

³ Royal Gwent Hospital, Aneurin Bevan Local Health Board, UK

⁴ Hywel Dda University Health Board, Withybush General Hospital, UK

⁵ Princess of Wales Hospital, Abertawe Bro Morgannwg University Health Board, UK

⁶ Wrexham Maelor Hospital, Betsi Cadwaladr University Health Board, UK

⁷ University Hospital of Wales, Cardiff and Vale University Health Board, UK

Background: Approximately 1 in 17 people is affected by a rare disease in the UK, many of which are genetic in origin. Using DNA sequencing, these and many other conditions will soon be diagnosed antenatally or early in life, having major implications on paediatrics in terms of diagnosis, treatment, and prognosis. Many European governments are investing in Genomic Medicine and paediatricians are well placed to recognise these new opportunities and integrate them into their practice.

Objective: In 2017 the Welsh Government introduced the Genomics for Precision Medicine Strategy, aimed at developing genetics and genomics to improve health and healthcare provision in Wales. To help fulfil this strategy, Swansea University launched an MSc in Genomic Medicine in May 2018, the first University in Wales to provide this opportunity to multi-disciplinary health care professionals, imparting the skills and knowledge necessary to interpret genomic data and understand its impact on patient care.

Methods: Based on the indicative curriculum of Health Education England and Genomics England, this 2 year part-time Masters course integrates lectures, workshops, tutorials, interactive group learning and guest lectures, while encouraging and facilitating workplace learning. Modules include bioinformatics, genomics of common and rare inherited diseases, ethics, techniques and optional modules such as pharmacogenomics. Module assessments are summative and students must complete a dissertation describing an applied genomics project, research, or literature review.

Results: Eight students completed their first year of study in January 2019. A variety of healthcare professionals enrolled (e.g. laboratory-based microbiologists, geneticists, pathologists, oncologists and paediatricians) which fostered collaborative work and learning. The formal and informal feedback to all modules has been excellent, with students ranking all aspects as “highly satisfactory”.

Conclusion: The MSc in Genomic Medicine plays a vital role in educating healthcare professionals seeking knowledge and skills in this emerging discipline.

338

Genetic & Metabolic

49XXXXY Syndrome: A Case Report of a Rare Genetic Disorder

Sara El Badri ¹ , Nuha Elmamoun ¹ , Babu Paturi ¹

¹ Paediatrics, Our Lady of Lourdes Hospital, Drogheda, Ireland

Introduction: Boys with 49XXXXY have an additional three X chromosomes, giving them a total of 49 chromosomes. The most common features of 49XXXXY syndrome include: learning difficulties, speech difficulties, hypotonia, underdeveloped sex organs and infertility. The diagnosis can be made by karyotyping in cell samples taken from placental tissue by chorionic villus sampling, or from amniocentesis. CGH array is another method for diagnosis of 49XXXXY.

Case Summary: T.O. is an 11 months old male infant born at 36+1 weeks` gestation by elective C/S for severe IUGR, prematurity and breech. His mother is a 26 years old Irish lady, and she had no antenatal issues. His birth weight was 2.28kg. He was admitted to NICU after delivery for mild respiratory distress. On examination he was hypotonic and had micropenis. On day of life (DOL) 2 his sodium level was found to be 147, and continued to be raised until DOL 3 with high serum osmolality. He had a cranial USS on DOL 3 which showed several sub-ependymal cysts in the frontal lobe suspicious for a congenital cyst formation. He also had a renal USS which was normal.

At 4 weeks of age he had an MRI Brain which showed connatal cysts adjacent to the frontal horns of both lateral ventricles.

He was discharged on DOL 16 from the NICU. He had 4 monthly pediatric outpatients follow up as well as physiotherapy follow up.

T.O. had recurrent chest infections requiring admission. On his admission at 8 months of age he had genetic and metabolic work up, and a diagnosis of 49XXXXY was reached. An early intervention referral was made for his persistent central/peripheral hypotonia and his developmental delay.

Conclusion: Rare diagnosis like 49XXXXY can often be missed or diagnosed late due to lack of specific features. Features such as underdeveloped genitalia and hypotonia should always raise concerns of underlying cause.

276

Genetic & Metabolic

Hypobetalipoproteinemia: Two Clinical Cases

Ana Esteireiro ^1,2 , Blanca Sánchez ² , Miriam Ortega ² , Fabiola Guerrero ² , José Rivero ² , María Ruíz ²

¹ Pediatrics Department, Caldas da Rainha Unit, Centro Hospitalar do Oeste, Portugal ² Adolescence Unit, Hospital Universitario La Paz, Spain

Background: The familial hypobetalipoproteinemia (FHBL) belongs to a heterogeneous group of monogenic diseases characterized by plasma levels of LDL cholesterol and apolipoprotein B (apoB)

Clinical Cases: Female of 12 years (Patient 1) and male of 20 years (P2) treated in the unit of Adolescent Medicine. In control analytics:

P1: Total cholesterol 85 mg/dL, LDL Cholesterol 37 mg/dL, apoB 26 mg/dL. GOT: 30UI/L, GPT 48 UI/L, GGT 17UI/L.

P2: Total cholesterol 66mg/dL, LDL 13mg/dL, apoB 26mg/dL, GOT: 25 IU/L, GPT 50UI/L, GGT 38UI/L. Ultrasound: diffuse increase in echogenicity compatible with steatosis.

Pending results of parental lipid screening and genetic study in both patients.

In both cases, secondary causes of hypolipemic were excluded.

Conclusion: LDL low cholesterol forces you to rule out secondary causes of hypolipemia. FHBL is due to mutations in the gene apoB or with less frequency of PCSK9 although in 50% of the cases does not become affiliated with any mutation. Diagnosis is important to prevent the emergence of homozygous FHBL in future generations, which is associated with degenerative neuromuscular diseases and retinal problems.

555

Genetic & Metabolic

Noonan Syndrome: A Syndrome with Wide Clinical Spectrum

Sofia Simões Ferreira ¹ , Liliana Sá ² , Joana Grenha ¹ , Isabel Pinto Pais ¹ , Andreia Teles ¹ , Helena Santos ¹ , Fátima Santos ¹ , Ana Luisa Leite ¹

¹ Department of Pediatrics, Vila Nova de Gaia/Espinho Hospital Center, Portugal ² Department of Pediatrics, Entre Douro e Vouga Hospital Center, Portugal

Noonan syndrome (NS) is a genetic disorder with an estimated incidence of 1/1000–2500 live births. Owing to multiple organ involvement, a wide spectrum of symptoms and signs may occur in this syndrome as well as a high variable expressivity.

We present two cases of NS with different presentations.

4-year-old boy, with prenatal diagnosis of cystic hygroma, pleural effusion and bilateral hydronephrosis. Amniocentesis was performed at 21 weeks for extended genetic and infectious study including Noonan Syndrome and Noonan spectrum mutation panel, which was negative. Admitted at birth due to prematurity of 32 weeks. During hospitalization, craniofacial dysmorphisms (CD) suggestive of NS were observed as well as multiple organ involvement hence he performed genetic study detecting a pathogenic heterozygosity variant c.1391GT (p.G464V) exon 11 in the BRAF gene. He is currently medicated with propanolol for hypertrophic cardiomyopathy. Maintains delayed psychomotor development and feeding problems, exclusively fed through gastrostomy and growing below -2SDS (NS Growth Charts).

16-year-old girl, referred to hospital consultation at 3 years of age due to CD. Performed a metabolic study, karyotype, muscle biopsy and mitochondrial DNA study that were normal. Subsequently she abandoned follow-up and was referral again at age 13 due to pubertal delay and short stature. She presented CD, unilateral mammary bud, very scarce pubic hair, pulmonary stenosis, syndactyly, interventricular comunication, pielic ectasia and intellectual disability. NS was suspected and confirmed by the finding of a heterozygosity variant c.4AG(pSer2Gly) in the SHOC2 gene.

The severity of NS varies widely, from presentations that are lethal prenatally to mildly affected individuals. Because presentation can be mild and the typical facial features recede with age, the diagnosis might be overlooked, while in severe cases the diagnosis is made sooner and multidisciplinary approach is often needed to address the medical and developmental complications.

1059

Genetic & Metabolic

Stickler Syndrome, a Genetic Inheritance: Case Report

Joana Filipe Ribeiro ¹ , Íris Santos Silva ¹ , João Virtuoso ¹ , Leonor Salício ¹ , António Mendes ¹

¹ Department of Pediatrics, Hospital de Sousa Martins - ULS Guarda, Portugal

Background: Stickler syndrome (SS) is a connective tissue disorder affecting the normal collagen production. Majority of cases present an autosomal dominant inheritance pattern. Clinical presentation is characterized by ocular (myopia, vitreoretinal degeneration and retinal detachment), skeletal (premature osteoarthritis, mild spondyloepiphyseal dysplasia and joint hypermobility) and orofacial findings (midfacial underdevelopment, cleft palate and micro/retrognathia), along with neurosensorial hearing loss.

Case Report: A 5-years-old female child was born from a supervised gestation in which a mandibular hypoplasia was suspected in the echography screening of the 2^nd trimester (16 weeks). For this reason, she underwent prenatal testing which showed a normal karyotype (46XX) with no further abnormalities. Her father had a previous diagnosis of SS (molecularly confirmed) displaying ocular and osteoarticular manifestations. More recently a patient’s brother was also diagnosed with SS in a prenatal genetic screening. Our patient was born from a full-term pregnancy with an Apgar index of 8/9/10. On examination, she presented a Pierre Robin sequence and conventional mechanical ventilation was needed during the first 72 hours due to airway obstruction. The patient was hospitalized during 24 days for feeding support. At 12 months of age she was submitted to a surgical correction of cleft palate with a favourable clinical outcome. Subsequent ophthalmological examination depicted a high-grade myopia.

Taking into consideration the presence of a Pierre Robin sequence, high-grade myopia, joint hypermobility (Beighton score of 8/9) and positive first-degree SS family history, the diagnosis of SS was established and posteriorly confirmed with a genetic study. The patient is currently under a multidisciplinary follow-up including speech therapy and psychology.

Conclusion: SS diagnosis is clinically based, encompassing variable phenotypic expressions depending on the inheritance pattern. In the presence of a Pierre Robin sequence a high index of suspicious is needed for the diagnosis of SS, which requires a multidisciplinary management and follow-up.

1043

Genetic & Metabolic

Cri du Chat Syndrome: Case Report

Catarina Freitas ¹ , Paula Rendeiro ² , Maria José Costa ³

¹ Serviço de Pediatria, Hospital Pedro Hispano, Portugal ² Centro de Genética Clínica, Portugal ³ Serviço de Neonatologia, Hospital Pedro Hispano, Portugal

Background: Cri du Chat or cat cry syndrome is a rare disorder resulting from a deletion, of variable size, on the short arm of chromosome 5 (5p-). The incidence is estimated between 1:15.000 and 1:50.000 live-born infants. Literature data report 10% of mortality, 75% of which occurs within the first year. The most important clinical features are the high-pitched cat-like cry early on life (apparently related to vocal cord abnormalities) as well as low birth weight, microcephaly, round face, broad nasal bridge, epicanthal folds, micrognathia and psychomotor delay and intellectual disability. Moreover, other malformations, although not very frequent, may be present: cardiac, neurological and renal abnormalities. With advancing age, the clinical manifestations could become less striking, making diagnosis more difficult.

Clinical Case: A female infant, of 8 months, institutionalized, with unknown parents, present from the birth high-pitched cat-like cry, broad nasal bridge, and microcephaly, being followed in consults of neonatology. According to clinical records, it was a single term pregnancy, with normal evolution and eutotic delivery with Apgar Index 9/10. Given the suspicion of Cri du chat syndrome, a genetic study (FISH) was done that was negative for 5p. Then, the chromosomal array study (aCGH) was performed, which revealed deletion in the terminal region 5p, confirming the possibility of the etiology of the patient`s phenotype.

Conclusion: Although there is no specific therapy for this syndrome, this case highlights the importance of timely diagnosis for effective educational and rehabilitation interventions, encouraging the performance of the genetic study, namely the chromosomal array study. Moreover demonstrates that make a diagnosis for a genetic or rare disease can often be challenging and thus the valuation of the clinical findings is very important.

220

Genetic & Metabolic

Newborn Screening Pilot Study Showed a High Incidence of Cystic Fibrosis in Republic of North Macedonia

Stojka Fustik ¹ , Violeta Anastasovska ² , Dijana Plaseska Karanfilska ³ , Lidija Spirevska ¹ , Ana Stamatova ¹

¹ Department for Cystic Fibrosis, University Children’s Clinic, Macedonia ² Laboratory for Newborn Screening, University Children’s Clinic, Macedonia ³ Research Center for Genetic Engineering and Biotechnology “Georgi D. Efremov”, MANU, Macedonia

Background: Cystic fibrosis (CF) is the most frequent, life limiting, genetic disease in Caucasians, caused by mutations in the CFTR gene. The incidence varies in different geographical areas and ethnic groups. The last two decades newborn screening (NBS) for CF has been implemented in the majority of countries where CF is common as a well-established public health strategy.

Objective: In May 2018, NBS for CF was introduced in R.N.Macedonia as a pilot study (in 13out of 30 maternities), included in National program for mothers and children’s care of the Ministry of Health. Methods: Two steps IRT-IRT algorithm were performed, and then a sweat test for confirmation/exclusion of the CF diagnosis when IRT values were both over the cut off. In cases of positive or borderline sweat tests, mutation analysis of CFTR gene were performed (snapshot reaction for 11 most common regional CFTR mutations or extended gene analysis).

Results: During the period of 6 months 9332 newborns were screened for CF. For IRT1 the cut-off level of 70 ng/ml was established (99.5th percentile) and for IRT2 a fixed cut off was used at 40ng/ml. Out of 11 screening positive cases, diagnosis of CF was confirmed in 5 (sweat test results: 90. 102..101..106..102mmol/L). All diagnosed cases at the end of the first month of life, already had symptoms consistent with the diagnosis of CF. The patient`s genotypes were: F508del/F508del (3), F508del/G1349D and F508del/G542X. In the same period, two symptomatic cases were diagnosed (mean age of diagnosis 4.5 months), born in maternities where the pilot study was not conducted (genotypes: F508del/R1158X and F508del/CFTRdel2,3).

Conclusion: The pilot study showed a high incidence of CF in our region of 1:1860 live births. From 2019, NBS for CF will be implemented on the whole newborn population in the country, since early diagnosis of CF through NBS and the appropriate preventive and curative care management of affected children has a proven benefits with respect to clinical outcomes and health economics.

656

Genetic & Metabolic

Early Maternal Deprivation and Epigenetic Modifications of Later Childhood Emotional Status.

Ketevan Gogberashvili ¹

¹ Pediatrics, Tbilibi State Medical University, Georgia

Background.There is now well documented that environmental agents can modify health by disruption of the homeostatic regulatory mechanisms between the nervous, endocrine and immune systems. During early postnatal life the brain exhibits high plasticity which allows environmental signals to alter the trajectories of rapidly developing circuits. Adversity in early life is able to shape the experience-dependent maturation of stress-regulating pathways such as the hypothalamo–pituitary–adrenal (HPA) system.

Objective. Present study was performed to make clear the influence of early maternal deprivation on formation the emotional status in later childhood; To understand the effect of early deprivation on the regulatory mechanisms of HPA axis.

Methods. The blood neurotransmitters (Norepinephrine NE, Dopamine DA, Serotonin SE) levels were investigated in healthy institutionalized children at age 6 to 36 months from Tbilisi Infant’s Orphanage. Emotional status was assessed in institutionalized children at age 3 to 6 years by Leusher’s Color Test. Control group formed the same age healthy children from Tbilisi mother-child shelters.

Results. Elevation in blood NE and low levels of DA and SE levels were established in the institutionalized infants to compare with control group. The higher was the percentage of children with anxiety and behavioral disorders in the group of orphan children, than in children with family care.

Conclusion. In this study early life stress has been shown to be a strong predictor of impaired inhibitory feedback regulation of the HPA axis. Thus we have proposed that conditions of early life environment can evoke changes in DNA methylation facilitating epigenetic programming of critical genes involved in regulating stress responsivity that may in turn manifest with neuroendocrine and behavioral symptoms in later childhood periods. So, it is recommended to support alternative ways of abandoned children`s care against children`s institutions, such as biological mothers’ financial support or foster care.

797

Genetic & Metabolic

Diabetes Mellitus and Optic Atrophy: Not Just a Coincidence

Ana Gomes ¹ , Filipa Pinto ¹ , Joana Figueirinha ¹ , Sérgio Silva ² , Cláudia Melo ³ , Jorge Pinto Basto ⁴ , Soraia Tomé ¹ , Paula Fonseca ¹

¹ Department of Pediatrics, Centro Hospitalar do Médio Ave, Portugal

² Department of Ophthalmology, Centro Hospitalar de São João, Portugal ³ Department of Pediatrics, Centro Hospitalar de São João, Portugal ⁴ Laboratory of Molecular Diagnostics and Clinical Genomics, Centro de Genética Clínica e Patologia (CGC Genetics), Portugal

Wolfram Syndrome (WS1) is a rare autosomal recessive genetic disorder characterized by diabetes mellitus, diabetes insipidus, optic nerve atrophy, hearing loss and neurodegeneration. WS1 may be caused by variants in the WFS1 gene that encodes a protein called wolframin. A 10-year-old boy presented to the emergency department for dysuria, polyuria and polydipsia with four days duration. Recent changes in appetite or weight were denied. He had a history of syncope episodes and progressive vision loss since the age of 7 and was recently diagnosed with bilateral optic nerve atrophy. His physical exam was normal and he had a Tanner stage I. Capillary blood glucose measurement was 388 mg/dL and urine test strip presented glycosuria without ketonuria. His blood pH was 7.38, potassium level was 3.7 mEq/L and bicarbonate level was 27.2 mmol/L. He had a normal lipid profile and thyroid function, negative anti-thyroid antibodies, negative screening for celiac disease and negative anti-islet cell and anti-glutamic acid decarboxylase antibodies. Considering the combination of optic nerve atrophy and diabetes mellitus, a genetic study for Wolfram Syndrome was performed and revealed 3 variants: 2 of uncertain clinical significance and one probably pathogenic variant. He had a normal tympanogram and audiogram and abdominal ultrasonography did not reveal any abnormalities. A multiple dose insulin injection therapy was started and he was referred to diabetes and genetics consultation. WS1 has poor prognosis and currently there is no effective therapy for this syndrome. Regular audiologic evaluation will be essential to early identify hearing loss. Therefore, a timely recognition and diagnosis is essential in order to minimize the complications and reduce morbidity and mortality.

795

Genetic & Metabolic

Rhabdomyolysis’ Etiology: A Challenging Diagnosis For A General Pediatrician

Cátia Leitão ¹ , Isabel Ayres Pereira ¹ , Joana Tenente ¹ , Margarida Peixoto ¹ , Marta Vila Real ¹ , Helena Santos ¹

¹ Pediatric Department, Centro Hospitalar de Vila Nova de Gaia/Espinho, Portugal

Background: Rhabdomyolysis is a muscular necrosis with release of its constituents into the circulation. It can be a manifestation of multiple disorders of different origins. A thorough clinical history and laboratorial investigation, with a high index of suspicion are of paramount importance to an accurate diagnosis.

Case: A 6 year old female was admitted to the emergency department (ED) with fever and odynophagia. She had a previous history of intermittent toe-walking and creatine kinase (CK) elevation. A blood test in the ED revealed mild anemia, elevated CK, and serology compatible with Epstein-Barr Virus (EBV) infection. During follow-up, a past history of myalgia and exercise intolerance was found. The patient also presented gastrocnemius hypertrophy, negative Gowers sign and an otherwise normal neurological examination. Continued elevation of CK was detected, so she did baseline metabolic studies and Pompe disease screening with no alterations. A muscle biopsy revealed glycogen deposits and an absence of myofosforilasis, suggestive of McArdle disease, confirmed by the presence of c.148CT (p.Arg50*) and c.40delA (p.Ile14Serfs*12) variants in PYGM gene. Patient was started on a carbohydrate enriched diet and light to moderate aerobic exercise regimen, and information on rhabdomyolysis was given. A progressive normalization of CK levels and physical activity were observed.

Conclusion: McArdle disease, or type V glycogen storage disease is a rare, recessive autossomic pathology caused by a mutation on the PYGM gene, resulting in a myofosforilasis deficiency. Only 4% of the cases are diagnosed before the age of 10. Its clinical presentation and severity is highly variable, ranging from severe hypotony, gait abnormalities, exercise intolerance, myalgia and muscle fatigue. Patients usually present with elevated CK levels and episodic rhabdomyolysis, myoglobinuria and acute renal failure. This case highlights the importance of investigating rhabdomyolysis’ possible etiologies, in order to prevent or allow an early detection of acute decompensations.

825

Genetic & Metabolic

Down Syndrome – A Neurodevelopmental Perspective

Joana Lorenzo^1,2, Ariana Teles², Diana Gonzaga², Inês Vaz Matos², Ana Catarina Prior²

¹ Neurodevelopmental unit, Joana Lorenzo, Portugal

² Neurodevelopmental Unit, CMIN-CHP, Portugal

Background: Down syndrome(DS) is the genetic leading cause of intellectual disability. Its worldwide incidence is 1:700-800 pregnancies, with 10000-12000 estimated cases in Portugal. The main characteristics include physical features, specific medical conditions and neurodevelopmental disorders.

Objective and Methods: Retrospective and descriptive study, with the aim of analyzing patients with DS in a Neurodevelopment Unit of a Level III Hospital in 31/01/2019.

Results: 29 patients with DS were included, 3(10,3%) with prenatal diagnosis; median maternal age of 29 years; 18(62,1%) male gender, between 1-18 years-old (mean and median 7 years). The first neurodevelopment consultation occurred between 1 month-14 years old.

21(72,4%) patients had cardiopathy, 17(58,6%) endocrine, 16 (55,2%) respiratory, 7(24,1%) gastrointestinal, 2(6,9%) neurologic and 2(6,9%) haematological disorders.

Regarding neurodevelopmental assessment, all patients had communication disorders, 5(17,2%) presenting Attention Deficit Hyperactivity Disorder(ADHD) under methylphenidate and 1(3,4%) Autism Spectrum Disorder(ASD). Concerning interventions applied, 28(96,6%) attended speech therapy, 25(86,9%) occupational therapy and 9(31,0%) physiotherapy. The adopted strategies were based on the learn to read to learn to talk program, phonomimic method (Teles P, Portugal) and Dolman Method in 2 patients each and Makaton method in 1 patient. In the remaining the intervention method was not specified. 18 patients(62,1%) were schoolers (median 8,5 years), benefitting from inclusive education.Conclusion: This review presents a holistic understanding of DS. In our sample the referral to Neurodevelopmental consultation was late, when it should occur precociously with the remaining specialties. Diagnosing neurodevelopmental disorders in DS patients is challenging, since their learning disabilities, maturity delay and the syndrome itself. Therefore, an ASD diagnosis is often late, which worsens the outcome. Besides, most demonstrate attention deficit, restlessness and impulsivity, with ADHD in only 17,2% of our sample. The prognosis is influenced by the onset, type and frequency of the intervention. The ideal method should be multi-modal, flexible and focused on the subject, family and community.

308

Genetic & Metabolic

Burnside-Butler Syndrome: A Diagnosis in Neurodevelopmental Delay

Pedro Marinho ¹ , Ana de Carvalho Vaz ¹ , Mariana Bastos Gomes ¹ , Virgínia Sampaio ¹ , Mariana Costa ¹

¹ Pediatrics Department, Unidade Local de Saúde do Alto Minho, Portugal

The authors report a 15-year-old Caucasian male, with a history of learning/intellectual disability (very low verbal and full-scale IQ (<70)), combined type attention-deficit/hyperactivity disorder (ADHD), an unusual happy appearance, overweight, mild tricuspid valve regurgitation and facial pilosity asymmetry. Karyotype XY, 46 and PCR revealed a FMR1 allele of normal size. Through microarray analysis, he was identified with a copy number variation - a 537Kb deletion between BP1 and BP2 on 15q11.2, involving various genes, including CYFIP1, TUBGCP5, NIPA1 and NIPA2.

Patients with 15q11.2 BP1-BP2 microdeletion (termed Burnside-Butler) syndrome, according to recent literature, can present with developmental delay (speech delay, writing and reading difficulties, verbal IQ ≤75) and neurobehavioral abnormalities including ADHD and unusual happy expression; less commonly, patients present mild dysmorphic features and congenital heart defects. These features are present in our patient. However, overweight or obesity have not been directly associated. Specific involvement of facial pilosity or tricuspid valve defects have not been previously described in this emerging cytogenetic syndrome, whose phenotypic features are still in expansion.

The authors aim to further the awareness of this etiologic entity, believing that achieving a diagnosis offers a basis on which to delineate the approach both to the patient and to their families, who in this case were oriented to Medical Genetics evaluation.

837

Genetic & Metabolic

Partnering with Patients in the Creation of Online Resources for Interprofessional Education

Leona Nertney³, Daire O'Leary³, John Hayden¹, Helen French², Frances Horgan², Judith Strawbridge¹

¹ School of Pharmacy, Royal College of Surgeons in Ireland, Ireland

² School of Physiotherapy, Royal College of Surgeons in Ireland, Ireland

³ School of Medicine, Royal College of Surgeons in Ireland, Ireland

Rationale: Collaborative practice happens when healthcare professionals work together with patients and families to deliver the highest quality of care. This is the optimal model for paediatric care, with children and families included as partners within the healthcare team. Interprofessional education (IPE) is seen as a necessary step towards collaborative practice. We sought to explore how best to prepare our students for paediatric collaborative practice, harnessing the unique interprofessional learning opportunities in paediatric care. This also required navigating logistical IPE challenges such as asynchronous curricula, unequal class sizes and delivery across international campuses.

Methods: We collaborated with a parent in creating case-based online IPE. Videos of the parent/child in consultations with a paediatrician, pharmacist, physiotherapist and speech and language therapist were recorded and embedded into an interactive Articulate Storyline® package. Pre-post MCQs and key teaching points covered a range of issues including developmental stages, medication administration and complex communication.

Findings: Medical students are currently engaging with the online IPE on a rotational basis and student reaction to the learning is ongoing. Delivery in the pharmacy and physiotherapy curriculum is planned. Currently the learning outcomes for interprofessional collaborative practice are, therefore, obtained unprofessionally.

Discussion / Conclusion

The patient-centred online interprofessional activity has provided an opportunity to begin to prepare our students for paediatric collaborative practice. We intend to explore further progression towards learning with others and evaluate the outcomes at a higher level.

707

Genetic & Metabolic

Patient with De Novo Mutation of Waardenburg Syndrome

Eva Pedrero Segura ¹ , Silvia Gallego Gutiérrez ¹ , Anabel Valdivielso Martínez ¹

¹ Pediatrics, Primary Care Attention Málaga-Guadalhorce District, Spain

Background: Waardenburg syndrome (WS) is an auditory-pigmentary syndrome caused by absence of melanocytes. Melanin contributes to skin, hair, and eye color and it plays an essential role in the normal function of the inner ear. Based on the phenotypic or genotypic characteristics, WS is clasificated in four types. The prevalence of WS is estimated to be 1/42,000 and it is responsible for 1% to 3% of cases of profound congenital deafness.

Objective: Describing a clinical case of WS type I with de novo mutation of PAX3 gene that associates other malformations that are not included in this syndrome.

Method: A 2 years old boy with WS type I who associates small ventricular septal defect, epispadias and congenital scoliosis with two hemivertebra. These two last defects are not described in WS clinical features. He has three mayor WS criteria (dystopia cantorum, congenital sensorineural hearing loss, white forelock) and two minor criteria (synofrys and broad and high nasal root with narrow nose), also bilateral blue iris. He has a profound bilateral hearing loss so he wears headphones with bilateral ventilation tubes pending of cochlear implant. Nowadays he is also pending of correction surgery of epispadias. Our patient has a healthy 5 years old brother and parents without WS features. The genetic study of parents was normal so his mutation has arisen de novo in heterozygosis c.667CT (p.Arg222Ter) in PAX3 gene.

Conclusions: All patients with white forelock, hyperthelorism, iris heterocromia or distopia cantorum should be investigated for hearing loss. Patients with any malformation should be widely studied to rule out other associated malformations. The remarkable finding is that this patient associates abnormalities that we have not found associated with WS in the current literature.

922

Genetic & Metabolic

A Novel Pathogenic Mutation of Vitamin-D-dependent Rickets

Jacopo Norberto Pin ¹ , Micol Cossettini ² , Francesco Fabris ² , Stefano Martelossi ²

¹ Dipartimento di Salute della Donna e del Bambino, Università degli Studi di Padova, Italy ² UOC di Pediatria, Ospedale Ca' Foncello, Italy

Backgound: Vitamin-D-dependent rickets 1A (VDDR-1A) is an extremely rare, autosomic recessive genetic form of rickets caused by a defect in vitamin D 1α-hydroxylase enzyme which leads to low levels of 1,25-(OH) vitamin D. Herein, we report two Moroccan sisters R.E. and N.E. (respectively 3 and 15 months old), born from consanguineous parents, who presented with psychomotor retardation and failure to thrive.

Methods: Biochemical analyses in serum and urine were conducted using the automatic analyzer Cobas immediately after collection. Serum intact PTH was measured by chemiluminescence assay. 25-OH vitamin D and 1,25-(OH) vitamin D levels were measured by enzyme immunoassays.

The entire coding region of CYP27B1, CYP2R1 and VDR genes were sequenced through NGS technology. Relevant genetic variations were resequenced through Sanger technology. The in silico prediction models used were Polyphen2, SIFT, MutationTaster, PHRED, MutationAssssor.

Results: Both sisters showed typical laboratory findings of VDDR-1A including hypocalcemia, hypophosphatemia, elevated ALP and PTH. Further assesments demonstrated normal-elevated levels of 25-OH vitamin D with low 1,25-(OH) 2 vitamin D.

Radiological workup revealed osteopenia, widened and irregular epiphyseal plates and in the older sister rachitic rosary.

We therefore sequenced the entire coding region of genes that has been associated to this clinical condition. Both sisters showed the homozigotic mutation p.Leu169Pro (c.596TC) of CYP27B1 gene that has never been described before. All prediction models consider this mutation harmful.

Both sisters immediately started 1,25-(OH) Vitamin D supplementation (N.E. took a galenic formulation of Calcium too).

They both showed an adequate but incomplete treatment response with psychomotor retardation improvement, weight gain, normalization of serum Ca and P levels and a significant decrease of PTH and ALP levels.

Conclusion: We report a novel pathogenic mutation of CYP27B1 that leads to VDDR-1A. An early diagnosis is necessary to prevent serious complications of rickets that are only partially reversible.

951

Genetic & Metabolic

Piebaldism: A Cutaneous Pigmentation Defect

Sara Pinto ¹ , Lúcia Rodrigues ¹ , Rui Dourado ¹ , Márcia Gonçalves ¹

¹ Pediatria, Centro Hospitalar Vila Nova de Gaia/Espinho, Portugal

Introduction: Piebaldism is a rare inherited autosomal dominant disease caused by a mutation in the C-KIT gene located on chromosome 4q12-13. In the presence of this mutation, a defect occurs in the migration and subsequent proliferation of melanoblasts in some body areas during the embryonic development, causing acromy due to the absence of melanin production.

The most suggestive clinical finding is a triangular acromic area in frontal region associated with hair depigmentation (poliosis), present in 80-90% of the cases. Other areas such as limbs, chest and abdominal region may also be affected. Skin changes are present from birth and remain stable over time.

The differential diagnosis is made with other cutaneous pigmentation disorders, such as vitiligo, total albinism, Tietz Syndrome and Waardenburg Syndrome.

Case Presentation: A female newborn presented at birth with an acromic macula at the frontal region with associated local hair depigmentation and macular hypochromic lesions in previvial regions, forearms, and abdomen. Physical examination was otherwise unremarkable. She was born of an uneventful first gestation of 39 weeks, had good adaptation to extrauterine life and appropriate to gestational age somatometry at birth.

Familial history was positive for piebaldism (mother and maternal aunt). Ophthalmological and auditory evaluation were normal.

Currently she is 5 months-old and has adequate psychomotor and stato-ponderal development.

In the case described, the presence of circumscribed poliosis associated with areas of depigmentation in the limbs and abdomen since birth, the stable course and positive family history, helped in the diagnosis and differentiation from vitiligo. The remaining entities are excluded, taking into account the objective examination and auditory and ophthalmologic evaluations performed.

Conclusion: The conjugation of the clinical history, genealogical tree and objective examination is crucial to the diagnosis of this pathology, obviating the need for complementary tests.

835

Genetic & Metabolic

Gangliosidosis: Diagnosis and Management Challenges

Andreea Madalina Popescu ¹ , Alina Burcuta ¹ , Bianca Gabriela Olaru ¹ , Alexandra Faleschini ² , Laura Carasava ^1,3 , Carmen Magdalena Burloiu ²

¹ Pediatric, Grigore Alexandrescu Children's Emergency Hospital, Bucharest, Romania, Romania

² Pediatric, Alexandru Obregia Clinical Hospital of Neurology and Psychiatry, Pediatric Department, Bucharest, Romania, Romania

³ Pediatric, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania, Romania

Introduction: Gangliosidosis is an autosomal recessive lysosomal storage disease, expressed by excessive accumulation of gangliosides in central nervous system and reticulo-histiocytar tissue, caused by lysosomal hydrolysis deficiency.

Objectives: Gangliosidosis is a pathology that affects both neurologic and physical development with progressing regression, involving severe complication and unfavorable prognosis, requiring multidisciplinary collaboration for management and therapeutic attitude.

Case report: We present the case of a male patient aged 18 months, admitted to the Pediatric Neurology Clinic "Alexandru Obregia "for global developmental delay including cognitive and motor regression. He had multiple intercurrent respiratory infections during the first months of life, and at the age of 5 months was admitted in “Grigore Alexandrescu Children`s Emergency Hospital”, and at abdominal examination hepatomegaly was reported. After excluding other causes, giving the evidence of the medical history, the lysosomal enzyme activity was measured, highlighting the low value of betagalactosidase. After discharge of the hospital, the child had multiple admissions for respiratory intercurrences, presenting also cognitive and motor regression. Upon admission to the neurology clinic, the child was athrepsic, with dysmorphic features, generalized telengiectasis, proeminent abdomen, hepatomegaly, severe developmental disorder, generalized hypotonia, development level below 3 months of age. The ophthalmologic examination revealed the appearance of cherry red-spot. Cardiac ultrasound has not shown any changes at that time. Repeating enzyme dosing revealed low activity of betagalactosidase, which associated with clinical and paraclinic signs confirmed suspected gangliosidosis - most likely type I. In the following month the child presented cardiomyopathy requiring admission in teritorrial unit.

Conclusion: Gangliosidosis is one of the rare causes of global development disorder with regression in the first two years of life. Diagnosis is a challenge, requiring multidisciplinary collaboration. Prognosis is unfavorable because of the associated complications, with no curative treatment so far, life expectancy usually is only two years in type I gangliosidosis.

313

Genetic & Metabolic

Bloch Sulzberger Syndrome Case

Vira Semianchuk ¹ , Irina Parandii ² , Yaroslav Semkovych ¹

¹ Department of Children Diseases of Postgraduate Medical Education Faculty, Іvanо-Frankіvsk National Medical University, Ukraine

² Allergology department, Іvanо-Frankіvsk Regional Children Hospital, Ukraine

Background: Bloch Sulzberger Syndrome (incontinentia pigmenti) is hereditary X-linked disease with skin lesion and generalized ectodermal and mesodermal dysplasia.

The disease affects 1:91,000 children. It is caused by NEMO/IKK-gamma gene mutation, which is located on chromosome Xq28, however, the implication of mutation in Xp11 and Xq21 segments is not excluded in case with other phenotypes of that syndrome.

In the clinical course of the disease, 4 stages can be singled out: bullous or vesicular, hypertrophic, pigmental and atrophic.

Extracutaneous changes are found in 50% of patients. The most common are: dental anomaly (dentin deficiency or absence of a teeth row); visual organs disorders (cataract, vascular anomalies and profound changes of retina epithelium); CNS injury (spastic paralysis, convulsive disorder, mental retardation). Congenital heart disease, anomalies of skull, anomalies of kidneys, nail dystrophy, etc. occur less frequently.

Case Report: Eight-month-old girl was admitted in allergology department with numerous erythematic-pigmental and papular-bullous linear rash forming along Blaschko lines. Some bullous elements were covered with hard crust (photo 1). Clinical blood and urine analysis, biochemical blood test and immunogram showed no abnormality. Neurosonography, ultrasonic diagnosis and echocardiography did not find any pathology. The patient was initially diagnosed with multiforme bullous erythema. Differential diagnosis was made with herpes zoster, bullous impetigo, bullous mastocytosis. According to anamnesis, the first symptoms occurred in the maternity department, where the child was diagnosed and treated for herpes. Genetic confirmation is not available in Ukraine. However, classic clinical criteria confirm the diagnosis.

Today, the girl is 3 years old. CNS injury and visual organs disorders were not discovered. Bullous rash occasionally recur associated with respiratory condition. Pigmentation areas are present on skin along Blaschko lines (photo 2,3), as well as atrophic skin changes. There are areas of cicatrix alopecia on the skin of the head.

Conclusion: Although Bloch Sulzberger Syndrome is a rare disease, classic symptomatology is indicative of the right diagnosis. These children require case follow-up for early detection of optic nerve disorder and CNS injury.

706

Genetic & Metabolic

Anhidrotic Ectodermal Dysplasia: Different Fenotype in the Same Family

Ana Isabel Valdivielso Martnez ¹ , Eva Pedrero Segura ¹ , Silvia Gallego Gutierrez ¹

¹ Pediatrics, Primary care Attention, Spain

Background: Anhidrotic ectodermal dysplasia (AED) is a rare hereditary disorder with a triad os sparse hair, dental hypoplasia and anhidrosis. Most cases of AED are the X-linked form, while others are dominant o autosomal recessive form.

Objective: To report 4 cases of mild-moderate AED in four sisters, whose father also suffers from AED with the complete phenotype.

Methods: Clinical data were collected from a family with this disease.

Results: We report 4 cases of AED in four sisters. The genetical test revealed X-linked mutation of AED gen in all of them.

Case 1: 1 year old girl, she has had 2 bacterial pneumonias, recurrent wheezing and recurrent episodes of high grade fever. She has sparse hair, hypotricosis and small and pointed teeth. She is able to sweat.

Case 2: 2 years old girl, she has had 4 bacterial pneumonias, recurrent wheezing and recurrent episodes os high-grade fever. She has sparse hair, hypotricosis (image 1)and small and pointed teeth (image 2). She has very dry skin and she is not able to sweat, so it is very difficult to treat fever.

Case 3 : 3 years old girl, asimptomatyc.

Case 4 : 4 years old gir, she never had pneumonias but she has pointed teeth (picture 3) and sparse hair (picture 4)

Conclusion: the same genetic mutation presents with different grades of phenotypic alterations in the same family. It is important to suspect AED in children with rare teeth and sparse hair and freqent fever episodes.

1039

Genetic & Metabolic

The Language Difficulties Leading to Mucopolysaccharidoses

Joana Vilaça ¹ , Sofia Miranda ¹ , Anabela Bandeira ² , Clara Alves Pereira ¹ , Manuela Costa Alves ¹

¹ Paedriatic Service, Hospital de Braga, Portugal

² Paedriatic in the Metabolic Unit of the Reference Center for Inborn Errors of Metabolism, Centro Hospitalar do Porto, Portugal

The mucopolysaccharidoses (MPS) is a lysosomal storage disorder caused by the deficiency of enzymes required for the stepwise breakdown of glycosaminoglycans (GAGs). MPS is a rare condition, with an estimated total incidence of approximately 1 in 20000 live births. MPS type II or Hunter syndrome is a hereditary genetic disease linked to the X chromosome and is caused by the enzymatic deficiency of iduronate-2-sulfatase.

A 28-month-old boy, was admitted in paediatric with language difficulties. He has no relevant obstetric and family history. Normal growth with cephalic perimeter always in P95. Bilateral inguinal hernia surgery at 15 months. Chronical nasal obstruction with adenoid hypertrophy. Normal motor development with small delay in expressive language.

We observed macrocephaly, rough facial features (including a broad nose, thick lips and enlarged mouth and tongue), excessive body hair growth and hepatomegaly. The abdominal echography revealed hepatomegaly without splenomegaly or other alterations. Electrical and echocardiogram were normal. Without hearing loss or cornea opacity. The analytical study showed normal hemogram, lipid panel, hepatic and thyroid function. From the remaining investigation is important to emphasize the increased levels of GAG’s in urine excretion. He was sent to Metabolic Unit, where was confirmed and diagnosed the enzymatic deficiency of iduronate-2-sulfatase, MPS type II. He began enzyme replacement therapy at 32 months with regression of hepatomegaly and improved facial features.

The MPS is a lysosomal disease with large spectrum of presentation with severe types manifested in first year or attenuated forms with diagnostic in adult. There for it is necessary a high suspicion to do the diagnosis.

In the present case, the early diagnosis was possible due to a detailed clinical history and a meticulous objective examination. The early enzyme replacement therapy leads to a better quality of life whereas after the establishment of the sequels these are not reversible.

1047

Hemato – Oncology

Development and Validation of a Predictor Score of Persistent Thrombocytopenia in Primary Immune Thrombocytopenia in Children.

Habib Besbes ¹ , Chokri Chouchane ¹ , Radhia Hadj Salem ¹ , Saber Hammami ¹ , Islem Khefecha ³ , Bahri Mahjoub ² , Slaheddine Chouchane ¹

¹ Pediatric Department, LR12SP17, Fattouma Bourguiba Hospital Monastir University, Tunisia

² Pediatric Department,, Taher Sfar Hospital, Monastir University

³ Research Laboratory, Statistics, Economics and FinanceFSEG Mahdia, Monastir University

Aim: To develop a prognostic score predictive of persistent thrombocytopenia in acute primary immune thrombocytopenic (PIT) in children.

Methods: Two retrospective cohorts of PIT collected from two pediatric departments over a similar period of 25-year. One cohort served for the derivation of the score and the second served for the external validation. Inclusion criteria were: PIT age between 2 months and 14 years and persistent thrombocytopenia less than 100,000 / mm3 after 6 months of follow-up. The data was analyzed using statistical software SPAD version 5.5. The predictive index was done through a discriminant analysis.

Results: 219 cases were collected in the two studies, respectively: 149 cases (mean age = 5.7 years, sex ratio M / F = 1.48) from the derivation population and 70 cases from the validation population (mean age = 5.4 years, sex ratio M/F = 1.5). The developed score had 6 parameters: beginning onset, failure of the first line treatment, the presence of a triggering factor, age, platelet rate at 1 month and platelet rate at 3 months. The sum of the different coefficients for each patient provides a result convertible to probability of persistence of thrombocytopenia after 6 months. The ROC curve (Receiver operating characteristic) showed a good specificity (93%) and a good sensitivity of (90%) for a threshold of 35. The external validation study showed a good specificity e (100%) and good sensitivity (83.3%).

Conclusion: This prognostic clinical score validated by an external study requires further prospective studies to improve validation level and to explore its practical utility.

509

Hemato – Oncology

To “B” or not to “B”: Cervical Lymphadenopathy in a Previously Healthy 16-year-old Boy

Mafalda Castelão ^1,2 , Ana Raquel Claro ¹ , Pedro Silva ³ , Ana Barbosa Rodrigues ¹ , Ana Catarina Amorim ⁴ , Ana Isabel Fernandes ^2,5,6 , Daniela Macedo ⁵ , Isabel Esteves ^1,2 , Ana Mouzinho ^1,2 , José Gonçalo Marques ^1,2

¹ Department of Paediatrics, Hospital de Santa Maria, Centro Hospitalar Universitário de Lisboa Norte, Portugal

² Faculty of Medicine, University of Lisbon, Portugal

³ Department of Paediatrics, Hospital de Santo André, Centro Hospitalar de Leiria, Portugal

⁴ Department of Paediatrics, Hospital Fernando da Fonseca, Portugal

⁵ Oncoloy Service, Hospital de Santa Maria, Centro Hospitalar Universitário de Lisboa, Portugal

⁶ IMM, Instituto de Medicina Molecular João Lobo Antunes, Portugal

Background: Lymphadenopathy in children is common and generally benign and self-limited. Specific accompanying systemic symptoms can call for attention for a manifestation of serious underlying disease.

Case description: A 16-year-old male presented to the Emergency Department with a two-month history of a rapidly progressing nontender unilateral cervical mass, with no fever or other complaints. On thorough history, there was an objective weight loss of 8 percent of body weight and increased night sweats over the last few weeks. There were no complaints of pruritus and no past medical history of previous infections, exposures to toxins or evidence of underlying immune deficiencies or familial cancer. On physical examination a localized bulky, firm, rubbery, nontender lymphadenopathy in the lower cervical and supraclavicular region was found. There was no facial edema, dysphagia or dyspnea. He had a clear oropharynx and no evidence of hepatosplenomegaly or skin lesions. His blood tests revealed leukocytosis (22600/uL) with elevated C-reative protein (9,45mg/dL) and sedimentation rate (42mm/h). A CT scan revealed cervical lymphadenopathy in relation with a large mediastinal mass surrounding the aorta, causing complete obstruction of the superior vena cava (SVC) and significant compression of the trachea (axis 5mm). He underwent an excisional biopsy of the lymph node, diagnosing classic Hodgkin Lymphoma (HL). He was referred to Hematology to initiate treatment.

Conclusion: HL is the most common childhood cancer in the 15- to 19-year-old age group. Most patients present with only painless lymphadenopathy, usually cervical or supraclavicular. Seventy-five percent have a mediastinal mass at the time of presentation with risk of SVC syndrome. Nonspecific systemic symptoms including night sweats and weight loss, classified as "B" symptoms, are important indicators of malignant disease and warrant a detailed history and complete physical examination for early detection.

617

Hemato – Oncology

Human Papilloma Virus E7-loaded Fetal Macrophages Could Trigger E7-specific Th1 Immunity against TC-1 Tumorigenesis

Jeng-Chang Chen ¹ , Liang- Shiou Ou ¹

¹ Department of Surgery, Chang Gung Children's Hospital, College of Medicine, Chang Gung University, Taiwan

Background: In our previous study, fetal exposure to ovalbumin caused Th2-skewed immunity. It was attributed to fetal macrophages that sequestered endocytosed ovalbumin, differentiated toward dendritic cells and later instruct T-cells as they fully developed to more efficiently deal with antigens.

Objective: This study was aimed to examine whether fetal macrophages could elicit Th1 immunity against tumor cells.

Methods: Fetal phagocytes from gestational day 14 C57BL/6 fetal liver and peritoneal cavity were in vitro pulsed with human papillomavirus (HPV) E7 peptides overnight.Then, they were transferred intraperitoneally into 4-6 week-old C57BL/6 mice. One month later, recipient mice were assessed for their T-cell activation by IL-2 release and T-cell polarization by IL-4, IL-5 and IFNγ secretion in the culture system under HPV E7 stimulation using ELISA. Lymphocyte proliferation was also measured by the incorporation of tritiated thymidine. Recipients were further evaluated for their resistance to TC-1 tumor cell challenge by monitoring tumor growth and recipient survival following subcutaneous inoculation of 10⁵TC-1 cells.

Results: After adoptive transfer of HPV E7-loaded fetal macrophages,recipient lymphocytes exhibited the activity of lymphocyte activation and proliferation specifically in response to HPV E7 stimulation as evidenced by significant IL2 secretion and tritium incorporation. Recipient lymphocytes displayed a Th1-skewed phenotype as evidenced by significant secretion of IFNγ rather than IL-4 and IL-5 under HPV E7 stimulation. Adoptive transfer of HPV E7-loaded fetal macrophages sufficed to protect the recipients from TC-1 tumorigenesis with tumor-free survivals of 70% at 2-month follow-up, but failed to completely eliminate pre-existing TC-1 cells despite perceptible attenuation of local TC-1 tumor growth.

Conclusion: Following endocytosis of tumor antigens, fetal macrophages could trigger tumor antigen-specific T-cell immunity to preclude tumorigenesis. It shed light on the crucial role of fetal macrophages in tumor immune surveillance, thereby eliminating transformed cells egressing during embryogenesis.

368

Hemato – Oncology

Glucose-6-Phosphate Dehydrogenase Deficiency: A Case Series

Sofia Cochito Sousa ^1,2 , Nélia Costa ² , Cristina Trindade ² , Teresa Ferreira ² , Alexandra Dias ²

¹ Departamento de Pediatria, Hospital de Santa Maria – Centro Hospitalar Universitário Lisboa Norte, EPE, Portugal

² Departamento da Criança e Jovem, Hospital Prof. Doutor Fernando Fonseca, EPE, Portugal

Background: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common human enzyme defect and has a wide variety of clinical manifestations. The highest frequencies are seen in Africa, Asia and Mediterranean region, but with world globalization it`s becoming prevalent worldwide.

Objective: Characterization of a pediatric G6PDH deficient cohort followed in a suburban area of an European capital.

Methods: Retrospective study of children with G6PD deficiency in a Pediatric Hematology consultation (2013 to 2018). Record data: demographic, clinical and laboratory information.

Results: 34 children were included, most of them male (82%) and with African ancestry (74%). The median age at diagnosis was 1.1 years (IQR 28days-7.8years). Diagnosis was made after: acute hemolytic anemia episode (n=13;38%), neonatal hyperbilirubinemia (n=10;29%), family screening (n=6;18%), neonatal anemia (n=3;9%) or investigation of anemia (n=2;6%). Median enzymatic activity was 1.3U/gHb (range 0.3-4.8U/gHb; reference 5U/gHb) and there was no difference between gender. Concomitantly, 3 patients had alpha-thalassemia, 2 sickle-cell disease and 2 hemoglobin Hope.

76% had neonatal hyperbilirubinemia, 75% of these with phototherapy criterion and 1 needed exchange-transfusion; 35% had neonatal anemia and 41% had at least one episode of acute hemolytic anemia, 50% trigged by ingestion of fava beans and 43% by infection. At the acute hemolytic episode, mean minimum hemoglobin was 6.4±2.7g/dL and 50% needed blood transfusion.

All children were followed in hematology pediatric consultation: one has chronic hemolytic anemia and only one (with sickle-cell disease) had another episode of acute hemolysis, trigged by infection and immune process Mutation test was performed in a minority (n=6;18%).

Conclusion: In this study, neonatal period and infection were important triggers of hemolysis and high prevalence of females was found. We should keep in mind the possibility of concomitance of different causes of hemolysis and that early diagnosis is crucial to prevent future episodes of acute hemolysis and consequent morbidity.

655

Hemato – Oncology

Sacro-Coccygeal Teratoma (SCT) in a Pacific Island Nation

Douglas Greer ^1,2 , Benjamin Wagstaff ^1,4 , Rajay Rampersad ¹ , Celine Hamid ¹ , Rooney Jagilley ³ , David Croaker ^1,2

¹ Department of Paediatrics and Child Health, The Canberra Hospital, Australia

² Department of Paediatric Surgery, Sydney Children's Hospital, Australia

³ Department of Surgery, National Referral Hospital, Solomon Islands

⁴ Department of Surgery, Coff's Harbour Hospital, Australia

Background: SCT is a rare tumour most common in children with quoted textbook incidences ranging from 1 in 27,000 to 1 in 40,000 live births. Several recent series suggest a higher incidence close to 1/10,000. Since 2015 our unit has been providing paediatric surgical support services to a developing tropical nation, and we have also noted an incidence of approximately 1/10,000.

Objective: To review the incidence and details of our series of Pacific Island SCT.

Methods: Retrospective review of SCT cases 2015- April 2019 inclusive was performed, including patient demographics, diagnosis, operation performed, and histopathology (where available). Incidence was calculated using World Bank population data. Statistical analyses were performed using Stata©.

Results: In total 8 patients presented with SCT in the study period. Mean age was 1 year (range 0-4). Five (62.5%) were female. Four (50%) were type 1 SCT, with two Type 2, and two Type 3. All eight were surgically typical of SCT, and were treated with primary excision. One is lost to follow-up. Of the eight operated on, three have been brought to Australia, and five had their operations at home. Available pathology showed benign mature SCT in all. One report has been lost, and one is awaited. Given a local birth rate of 28.7 per 1000/year, the approximate incidence of SCT regionally was 1 per 9840 births.

Conclusion: SCT remains a rare tumour, but local real incidence may be higher. Given that our observed incidence fits with the higher recently published incidences, we ask whether there has been a real worldwide increase in incidence of SCT over recent decades, or whether there is significant regional variability in incidence. We incidentally note the benefits of links between rich and poor world health services.

492

Hemato – Oncology

Parotid Swelling: A Rare Presentation of Leukemia

Nelia Gaspar ¹ , Ana Barbara de Matos ¹ , Marlene Salvador ¹ , Filipa Dias Costa ¹ , Julieta Morais ¹ , Ana Teixeira ²

¹ pediatric service, Centro Hospitalar Médio Tejo E.P.E, Portugal

² pediatric service, Instituto Português de Oncologia Francisco Gentil E.P.E, Portugal

Background: Acute leukemia represents the main cause of neoplastic disease in pediatric age. The most frequent clinical presentation of acute lymphoblastic leukemia (ALL) are hepatomegaly and/or splenomegaly, lymphadenopathy, fever, anorexia, weight loss, asthenia and sweating. Although other forms of extramedullary involvement may exist, initial parotid infiltration is very rare.

Clinical Case: A previously healthy nine-year-old boy was admitted to the emergency department due to progressive bilateral pre-auricular swelling over the last 10 days, with no other associated systemic symptomatology. Bilateral exuberant and painless parotid swelling was observed, with hard consistency and adhesion to the deep planes, as well as submandibular adenopathy, the larger with 3cm, hard and painless, without other changes on clinical examination. Blood tests showed 27810/uL leukocytes [normal (N) 5000-15000/uL], 18080/uL lymphocytes (N 1500-7000/uL), hemoglobin 9,9g/dL (N 11,5-15,5g/dL), mean globular volume 80,2fL (N 72-84fL), mean corpuscular hemoglobin 26,9pg (N 25-33pg), platelets 111000/uL (N 150000-500000/uL), peripheral blood smear with atypical lymphocytes, erythrocyte sedimentation rate 9,2mm/h (N 0-10mm/h), LDH 567 IU/L (N

Conclusion: Bilateral parotid swelling constitutes a rare initial presentation form of acute leukemia. The present case underlines the importance of clinical suspicion of neoplastic disease in the presence of parotid swelling with atypical evolution and in the absence of associated inflammatory signs.

561

Hemato – Oncology

Classical Hodgkin Lymphoma: A Case Study of a Female Adolescent Presenting with Systemic Symptoms and Microcytic Anaemia

Catarina Granjo Morais ¹ , Ana Rita Curval ¹ , Joana Pereira-Nunes ¹ , Catarina Sousa ² , João Luís Barreira ¹ , Artur Bonito Vítor ¹ , Teresa Nunes ¹

¹ Pediatrics Department, Centro Materno Pediátrico, Centro Hospitalar e Universitário São João, Portugal ² Pediatric Oncology Department, Instituto Português de Oncologia do Porto, Portugal

Hodgkin lymphoma (HL) accounts for approximately seven percent of childhood malignancy. It should be suspected in a child with lymphadenopathy, systemic complaints and mediastinal mass. Differential diagnosis includes other malignant, infectious and inflammatory diseases.

A 12-year-old female, with a six-month background of 15 percent weight loss, night sweats and fatigue and a recent diagnosis of microcytic hypochromic anaemia (Hgb 8.2 g/dL), presented at our emergency department due to aggravated anaemia (Hgb 7.9 g/dL) after a 30-day trial of iron therapy. Respiratory, gastrointestinal and bleeding symptoms were denied. Dietary restrictions, sick contacts and family history of autoimmune diseases were excluded. On examination, she presented fever, pallor and no palpable peripheral lymphadenopathy or organomegaly. Initial laboratory results confirmed microcytic hypochromic anaemia refractory to iron therapy. Blast cells were not seen on blood film, nor other morphologic abnormalities except microcytosis and atypical lymphocytes. It was also apparent an increase of acute-phase reactants (ferritin 515.3ng/mL, ESR 118 mm/hour), without elevated faecal calprotectin. Abdominal ultrasound was normal, however chest radiography revealed a subtle mediastinal widening. CT scan displayed an anterior mediastinal conglomerate of lymph nodes (8.7 x 6.5 x 9 cm) and mild splenomegaly (12.6 cm). She was transferred to an oncology center and excisional biopsy revealed a classical HL. Bone marrow involvement was excluded on aspiration and biopsy and FDG-PET scan confirmed supra-diaphragmatic nodal involvement.

About three-quarters of paediatric HL cases display a mediastinal mass on chest radiograph at the time of presentation. Mediastinal widening should never be overlooked particularly in the setting of classic B symptoms (fever, night sweats and ≥10 percent loss within six months before diagnosis). It should also be noted that malignant diseases as HL can produce microcytic anaemia, which may be misinterpreted as iron deficiency. Prompt referral to an oncology center is mandatory for diagnosis, staging and management.

723

Hemato – Oncology

A Case of Severe Autoimmune Hemolytic Anemia

Maria Gutu ¹ , Ruxandra Vidlescu ¹ , Cristian Scurtu ¹ , Mihaela Smarandoiu ¹ , Diana Derewicz ^1,2 , Andreea Giurgiuveanu ¹ , Doinita Sfrijan ^1,2

¹ Department of Pediatric Oncology, "M.S. Curie" Emergency Children's Hospital, Romania

² Department of Pediatrics, “Carol Davila” University of Medicine and Pharmacy, Romania

Introduction: We present the case of a 5 months old baby with severe hemolytic anemia.

Case presentation: The infant was admitted through transfer from another hospital with the diagnosis of severe anemia (Hemoglobin (Hb) = 3.6 g/dl) - apparently with sudden onset, no infections or treatments administered within the last 7 days.

The clinical examination revealed a baby in poor clinical condition with intense pale skin, scleral icterus, tachycardia (150 -170 b/min), systolic murmur of 2/6 in the V-th parasternal left space, hepatosplenomegaly.

The blood analysis indicate severe autoimmune hemolytic anemia (Hb = 3.6 g/dl, with high reticulocyte count, hyperbilirubinemia, hypersideremia , positive Coombs test).

The blood transfusion was postponed (due to the presence of several classes of autoantibodies - IgG 3+; IgM 2+; C3c+/-; C3d3+) and treatment with corticoids was started (first intravenously with Methylprednisolone 30 mg/day, slowly tapered and then orally with Prednisone).

Under the treatment, the evolution was favourable - from both clinic (good general condition, normal skin colour, no jaundice or hepatosplenomegaly) and biologic point of view (with progressive increase of the hemoglobin until the normal value for the age, negative direct Coombs test and normal values of sideremia and bilirubinemia).

Conclusion: It is essential that patients with severe anemia be admitted in a department were they can be completely investigated in order to get the appropriate treatment.

539

Hemato – Oncology

Another Acute Media Otitis, Now What?

Miguel Labrusco ¹ , Joana dias, Joana Dias ¹ , Sara Almeida ¹ , Luis Rodrigues ² , Lígia Paulos ¹ , Paulo Oom ¹

¹ Department of Pediatrics, Beatriz Ângelo Hospital, Portugal ² Department of Otorhinolaryngology, Beatriz Ângelo Hospital, Portugal

Background: Rhabdomyosarcoma is the most common soft tissue sarcoma in children, however its location on the ear or temporal bone is quite rare. We present a case report of an uncommon clinical presentation of this disease.

Clinical Case: Male child, 6 years old, healthy, presenting right ear otalgy, homolateral face oedema and lip commissure asymmetry. A suggestive examination prompted an initial diagnose of an Acute Media Otitis complicated with facial palsy.

Oral treatment was started, initially with amoxicillin 90 mg/kg/day and prednisolone 2 mg/kg/day, after which, due to lack of improvement, with cefuroxime 40 mg/kg/day, and a new course of prednisolone.

Because of progressive worsening symptoms, a hospital admission was proposed for intravenous antibiotics (Ceftriaxone 100 mg/kg/day + Clindamycin 20 mg/kg/day) and corticotherapy (prednisolone 2 mg/kg/day) and further investigation.

Urgent Computerized Tomography of the ear showed a right erosive media and external otitis, with total opacification of the ear, and mastoid osteitis on various locations, and Magnetic Resonance revealed a bulky tumefactive lesion, involving the right temporal bone.

An exploratory tympanomastoidectomy was proposed, revealing a bulky obliterative polypoid lesion, that was partially removed, with pericortical, cortical, tegmen tympani and mastoid invasion from a polypoid-like neoformation submitted to biopsy. Anatomopathological examination, revealed a Rhabdomyosarcoma of the ear. Chemotherapy was started, with clinical improvement.

Conclusion: Rhabdomyosarcoma presentation symptoms can often mimic Chronic Suppurative Acute Media Otitis, making it a challenging, but important differential, as an early diagnosis can dramatically influence the overall survival rates.

486

Hemato – Oncology

Neurotoxicity Associated with Methotrexate: An Alarming Benign Entity

Cristiana Martins ¹ , Bruno Loureiro ¹ , Catarina Magalhães ¹ , Isabel Rodrigues ¹ , Mónica Costeira ¹ , Ana Maia ¹ , Cátia Sousa ¹ , Tereza Oliva ¹

¹ Department of Pediatrics, Portuguese Oncology Institute Porto, Portugal

Introduction: Methotrexate is an essential drug in the treatment of children with acute lymphoblastic leukemia diseases. In this population, neurotoxicity has a prevalence range between 2.3-15% and can be characterized by a broad range of signs and symptoms (from headache to stroke-like symptoms).

Case Report: We present a young girl (11-year-old) with lymphoblastic lymphoma (in phase IIA) who as a history of leukoencephalopathy, which mimicked stroke (a transient episode of mild right central facial paresis, right hemiparesis with flexion of the finger and ataxia) one week after treatment with high doses intravenous methotrexate. Brain magnetic resonance imaging revealed "probable hyperacute ischemic lesion in the territory of perforating arteries of the left middle cerebral artery"; cervical eco-doppler and Bubber test were normal; thrombophilia study at the beginning of treatment of oncologic was normal. The patient`s symptoms completely improve within two days without sequels and was medicated with antiplatelet agent. Ten days after a new administration of methotrexate, she presented with right-side hemiparesis and dysarthria. Again with full resolution. The patient was discharged home without neurological symptoms and continued to the maintenance phase under prophylaxis with destometrophan (20 mg, 3 times daily) without intercurrences.

Conclusions: Neurotoxicity associated with methotrexate is transient and most patients continue to receive this therapy without intercurrences. Symptoms can be treated previously by administration of destometrophan and it is important that healthcare professionals recognize this entity.

547

Hemato – Oncology

ALPS: The Rare Diamond among Lymphadenopathies

Inês Rosinha ¹ , Sónia Lemos ² , Lea Santos ¹

¹ Pediatrics, Centro Hospitalar do Baixo Vouga, Portugal

² Pediatrics, Hospital Pediátrico de Coimbra; Centro Hospitalar e Universitário de Coimbra, Portugal

Introduction: Autoimmune Lymphoproliferative Syndrome (ALPS) is a rare primary immunodeficiency belonging to the subgroup of immune dysregulation diseases. It’s a disorder of lymphocytic apoptosis’ mediators, with subsequent generalized chronic non-malignant and non-infectious lymphoproliferation, and expansion of an unusual population of T lymphocytes (double-negative T cells). Chronic lymphadenomegaly and/or splenomegaly and autoimmune cytopenias are common characteristics.

Clinical Case: Four-year-old male, without personal relevant background, observed in paediatric consultation, due to cervical lymphadenopathy detected by two years of age, exacerbated in infectious contexts and in the last six months. Splenomegaly noticed during an acute febrile episode. Without constitutional symptoms. Maternal family history: autoimmune haemolytic anaemia. At physical examination, he had elastic and mobile cervical bilateral adenopathy conglomerates and splenomegaly. Complementary diagnostic tests (in fasting and without disease): Cervical echography: multiple ganglionic formations probably of reactive nature. Abdominal ultrasonography: globous liver and splenomegaly, with multiple ganglionic formations adjacent to splenic thread and pancreas. Blood test: Haemoglobin 12,3 g/dL, Total leucocytes 6,4x10⁹/L, Platelets 144x10⁹/L (150-450), Peripheral blood smear “reactive lymphocytes and slight anisocytosis”, C-reactive protein 0,46 mg/dL, Cholesterol HDL 18 mg/dL (45), Triglycerides 207 mg/dL (≤90), IgA 197 mg/dL, IgG 2150 mg/dL (350-1620), IgM 20 mg/dL (30 – 265).Considering the diagnosis of ALPS, he was sent to a Tertiary Hospital’s primary immunodeficiency consultation. The subsequent study reveals: B12 8124 pg/mL (295-1769); Immunophenotyping 10% lymphocytes α/βCD3+CD4-CD8-; Genetic study: Fas deficit heterozygosity for mutation c.817CA (p.Q273K).

He completed two prednisolone cycles. Currently (7 years old), under sirolimus, with frank improvement in lymphadenopathy dimensions and hepatosplenomegaly.

Conclusion: This clinical case illustrates the importance of the high level of clinical suspicion regarding generalized chronic lymphadenopathies and/or splenomegaly. Despite ALPS’s non-malignant nature, the diagnosis may be quite challenging and requires long-term clinical surveillance, particularly because of the increased risk of malignancy.

840

Hemato – Oncology

Osler-Weber-Rendu, an Early Presentation

Cristina Rodrigues ¹ , Joana Vilaça ¹ , Ana Ribeiro ¹ , Joana Macedo ¹ , Helena Silva ¹ , Almerinda Pereira ¹

¹ Department of Pediatrics, Hospital de Braga, Portugal

Background: Osler-Weber-Rendu syndrome is a rare autosomal dominant disease characterized by telangiectasias and arteriovenous malformations, which may affect multiple organs. Symptoms develop over time and are more likely to appear in adulthood.

Case Presentation: Ten-year-old girl with a medical history of atopic dermatitis, Henoch-Schonlein purpura at 3 years of age and positive Phadiatop test for inhalant allergens. Her mother, uncle and maternal grandmother had Osler-Weber-Rendu syndrome. At the age of 8 presented frequent spontaneous epistaxis, several times a week, even while sleeping, without seasonal predominance. There were no visible telangiectasias and no anemia was detected. Right anterior rhinoscopy showed vascular ectasia. It was decided in Genetics appointment to perform molecular study, which confirmed the presence of the family mutation of the gene ACVRL1 C. 1232G A p.arg411Gln, at 9 years old. The results of abdominal and vesical ultrasound, electrocardiogram and echocardiography were normal. She had menarche at the age of 10 years. At 10 years old she was evaluated because of easy fatigability, mainly at afternoon. Bilateral vascular ectasias throughout septal mucosa were detected at anterior rhinoscopy. Blood tests indicated an iron deficiency anemia (Hb 10.1 g/dl, with presence of target cells in peripheral blood smear and ferritin 3 ng/ml). She started iron oral supplementation 6 mg/Kg/day. One month later control hemogram indicated an hemoglobin rise to 13.3 g/dl and ferritin 16 ng/mL. Actually she is waiting for a contrast echocardiogram, brain angio-magnetic resonance imaging and new abdominal ultrasonography for arteriovenous malformations screening.

Discussion: Epistaxis is the most common symptom of the disease. If it appears in pediatric age, it is typically after 10 years old, and rarely requires invasive interventions to control bleeding. This child presents an exuberant phenotype, and therapeutic experience is scarce in the approach of the pathology at such an early age.

1046

Hemato – Oncology

A Rare Case of Neuroblastoma Presenting with Skin Findings

Sirin Sonmez ¹ , Uzuner Selcuk ¹ , Anil Gulsel BahaliI ² , Fatma Betul Cakir ³ , Tahaoglu Irmak ¹

¹ Department of Pediatrics, Bezmialem Vakif University, School of Medicine, Turkey

² Department of Dermatology, Benzmialem Vakif University School of Medicine, Turkey

³ Department of Pediatrics, Division of Pediatric Hematology and Oncology Disease, Benzmialem Vakif University School of Medicine, Turkey

Neuroblastoma is the most common extra-cranial solid tumor in infants and children, Neuroblastoma tends to behave quite differently in infants as compared to children over 12-18 months.

We present a rare case of neuroblastoma presenting with skin rash as a preliminary finding.

Case:

A 39-day-old male patient with no medical history was admitted to our hospital with yellow bullous rash that started on the face and spread throughout the body in 15 days. The patient was born at term by caesarean section. His neurologic development is appropriate for his age. There is no family history and his parents are not related. On physical examination, he had yellow bullous rash all around his body , inspiratory stridor was auscultated and respiratory distress was seen.

The patient was consulted to dermatology clinic for his bullous rash and skin biopsy was performed. Bullous pemfigoid was detected pathologically, which is rare condition in infancy. Because the patient had inspiratory stridor and respiratory distress chest X-Ray was performed and reported normal. For further evaluation the patient was consulted to pediatric pulmonary specialist and with their suggestion thorax CT was performed. A solid mass that was suspected to be a neuroblastoma was found. So we performed abdominal MRI, and taken tru cut biopsy and determined neuroblastoma. İmmunoglobulin levels and lymphocyte subgroups were analyzed and found normal due to suspicion of immune deficiency.

Conclusion: Rashes are extremely common in infancy. Most of them are innocent and transient. However, serious infectious, congenital skin diseases and sometimes malignant tumors should be kept in mind when dealing with a rash in infancy.

We recommend that every infant with a skin rash and respiratory distress should be investigated for a infancy neuroblastoma.

The clinician should keep in mind that skin rash may be the first clinical finding of neuroblastoma.

288

Hemato – Oncology

Dental Morbidity in Children with Iron Deficiency Anemia

Svetlana Tuleutayeva ¹ , Bibigul Abeova ² , Zhadyra Ashirbekova ³ , Kulzhakhan Shauyenova ⁴ , Aigul Kurdabayeva ⁵

¹ Dentistry, Medical university of Karaganda, Kazakhstan

² Children Diseases, Medical university of Karaganda, Kazakhstan

³ Dentistry, Medical university of Karaganda, Kazakhstan

⁴ Dentistry, Medical university of Karaganda, Kazakhstan

⁵ Dentistry, Medical university of Karaganda, Kazakhstan

Study of the dental disease prevalence in children with iron deficiency anemia (IDA).

Objective: to study the prevalence of caries in children with type iron deficiency anemia

Materials and Methods: A study of 1,500 people of children and adolescents in rural areas of Karaganda region aged from 3 to 17 years with IDA and dental diseases was conducted. 765 (51%) of them are boys and 735 (49%) are girls. Total number of children with IDA was 812 (54% of the total number surveyed). Mild IDA was in 288 (35.5%) children, moderate - in 181 (22.3%), severe - in 51 (6.4%).

Results: The prevalence of caries was 69.4% in patients with anemia, the prevalence of periodontal disease was 39%, dental-anomalies occurred most of all in children with anemia - 96,1%. The highest prevalence of caries in children with anemia was observed in preschool (73.9%) and younger school age (77.2%). In IDA, a mildly chronice form of caries was found in 54.3% of cases, the subacute form of caries was 9.51%, and the acute form - in 0.84%. With moderate IDA 39.1%, 14.8% and 12.2% respectively. When severe IDA - 32.4%, 32.4% and 19.1%, respectively. With an increase in the severity of IDA, a dynamic increase in caries was observed, indicating a direct correlation.

Conclusions: The prevalence of caries in 3-6 year old children on the background of IDA is 75.6% and without IDA - 71.3% (p 0.001), in 7-11 year old children 79.2% and 74.9% (p 0.001), in 12-17 year old children - 52.6% and 52.6% (p 0.001), respectively.

685

Infectious Diseases

Predicting Factors for Hospitalization due to Odontogenic Infections in the Pediatric Population

Mary Abdo ¹ , Michael Joachim ² , Said Abozaid ¹ , Yasmine Ghantous ² , Wael Nasser ¹ , Imad Abu El Na'aj ²

¹ Department of Pediatrics, Baruch Padeh Medical center, Poriya affiliated to the Faculty of medicine in the Galilee Bar-Ilan University, Israel

² Oral and Maxillofacial Surgery Department, Baruch Padeh Poriya Medical Center, Israel

Background: Orofacial infections of odontogenic origin are a common and important health concern in the pediatric population. Incorrect or late treatment may lead to serious and possibly life threatening complications.

Objective: The aim of this study is to analyze the data on pediatric hospitalizations due to odontogenic infections and to isolate parameters predicting prolonged length of stay (LOS).

Methods: Data was collected from the records of patients (age 15 years) who were hospitalized due to odontogenic infections in Pediatric/ Oral and maxillofacial surgery department in Baruch Padeh Poriya Medical Center- Israel between 1/2010-12/2015. Facial infections of other origin was excluded.

Results: A total of 411 patients were included. 58.7% of them were males, 66.9% of non-Jewish ethnicity. Roughly half were discharged after one day of hospitalization. 42.8% of patients were not previously treated with antibiotics. 79.8% of patients did not present 38° body temperature at admission and 98.8% of them were treated by drainage with or without extraction of the involved tooth. 91% of patients received antibiotics after discharge and 4.6% were admitted for rehospitalization. The calculated OR for odontogenic infections in patients from Arab background was 2.5 significantly higher (p0.0001) than their Jewish counterparts. The mean age in the study was 6.53±2.59 years. There was significantly higher (P0.05) values of White Blood Cells (WBC) and platelets in patients with 3 days LOS, patients with 3 days LOS were significantly older (8.36 vs 6.28) and OR for a prolonged LOS in Arab patients was 2.55 comparing to Jewish patients (p=0.01).

Conclusion: Risk factors for prolonged LOS were: older age, Arab ethnicity and higher WBC count and platelets values at admission.

878

Infectious Diseases

Viral Pathogen Association with Community Acquired Pneumonia in Hospitalized Children- Report from the State of Qatar

Manasik Hassan ^1,2 , Amal Alnaimi ² , Aji Varughese ¹ , Magda Youssef ² , Ahmed Alhammadi ²

¹ Peditaric, Hamad Medcial Corporation, Qatar

² Peditaric, SIDRA Medicine, Qatar

Background : Community acquired pneumonia is the leading cause of childhood morbidity and mortality globally, although the etiology of CAP varies by age, viruses remain the most etiologic agent and may lead to sever disease especially in children with co-morbidities. Data on community acquired pneumonia in children were found to be limited in Qatar.

Objectives: This study aimed to determine common virus associated with CAP in hospitalized children and to identify risk factor for pediatric intensive care unit (PICU) admission.

Methods: A cross-sectional single institutional retrospective descriptive study was performed at Hamad Medical Corporation, the only tertiary and academic medical center in the State of Qatar. The study included children admitted with community-acquired pneumonia from Dec 2017-June 2018, details of demographic and respiratory viral as detected by molecular assays were analyzed

Results: Total of 59 hospitalized children with diagnose of CAP between 3 months-14 years were included,mean age at diagnosis 3 years.43 patients (73%)had positive PCR nasal swab,single virus isolated in 31case,multiple viruses in 12 cases. The most isolated virus was Rhinovirus(21%),Human Meta-pneumovirus(17%),Boca virus(15%),H1N1influenza virus(8%),Respiratory Syncytial Virus and corona viruses were the least common with(7%) each. Nearly 20/59(33%)of children admitted to PICU,15(25%) had positive viruses in which the most frequent were H1N1influnza and Rhinovirus with longer PICU stay (median 10 days). Common risk factor associated with PICU hospitalization;prematurity and bronchial asthma.

Conclusions: Our study revealed that Rhinovirus and HMP Virus were the most common causative agents in hospitalized children with CAP. This finding in contrasts with a previous report from WHO in which RSV was the most frequently isolated virus. H1N1influanza virus play a major role in PICU admission, we need to increase public and healthcare providers knowledge to annual vaccine.

213

Infectious Diseases

The Journey to the Pediatric Intensive Care Unit Following Vaccine Refusal

Nukhet Aladag Ciftdemir ¹ , Rıdvan Duran ¹ , Tuba Eren ¹ , Mustafa Torehan Aslan ¹

¹ Department Pediatrics, Trakya University Faculty of Medicine, Turkey

Background: Vaccines are the most effective public health interventions against many vaccine-preventable diseases. In recent years, the number of parents refusing recommended childhood vaccination increased significantly. The concept of increasing vaccine refusal has emerged in the world in the 1990s and in Turkey in the beginning of 2010. Here, we report a clinical case of an infant with severe pertussis with vaccine-refusing parents.

Case Presentation: A 13 months-old girl was admitted to emergency department with a 10-day history of cough, which was gradually progressive. She was diagnosed with viral upper respiratory tract infection in another hospital. On examination, body temperature was 38.2^c, oropharynx was hyperemic and bilateral crackles were detected by lung auscultation. The remaining findings on her physical examinations were normal. Laboratory investigations showed the following results: WBC: 66.900/mm3 (80% lymphocyte), CRP negative, ESR 19mm/h, blood biochemistry, liver and kidney function tests were normal. Bilateral infiltrations were seen on chest radiography. Patient was diagnosed with pneumonia, and treated with intravenous ceftriaxone. Pertussis was suspected because of the infant`s persistent cyanotic cough and apnea, intravenous clarithromycin was added to antibiotherapy. Two days later, her WBC count had increased to 79.800/mm3 (85% lymphocyte). No tumor cells was detected in peripheral blood smear. She was transferred to the pediatric intensive care unit with, generalized tonic seizure after coughing. Brain computed tomography examinations and EEG were normal. The culture of nasopharyngeal secretions and polymerase chain reaction (PCR) were both positive for B. Pertussis. During his follow-ups, on 14th day of hospitalization, her WBC count gradually dropped, physical examination was normal and she was discharged from the hospital. The patient had been given all the missing vaccinations in the follow-up after discharge of the baby as a convinced family vaccination after discussing her illness with her parents and explaining all the facts of the event.

Conclusion: Children of parents who refuse immunizations are at increased risk of in for vaccine-preventable diseases. Vaccine refusal also increases the risk for the whole community. All parents should be informed about the benefits of vaccination and risk of severe infection deseases.

466

Infectious Diseases

Intermittent Irritability in a Toddler

Susana Alexandre¹, Pedro Fernandes³, Ana Mouzinho², Mafalda Castelão², Catarina Gomes¹, Sara Santos¹

¹ Paediatrics, Centro Hospitalar do Oeste - Caldas da Rainha, Portugal

² Paediatrics - Infecciology Unit, Centro Hospitalar Lisboa Norte - Hospital Santa Maria - Centro académico de Lisboa, Portugal

³ Orthopedic Unit, Centro Hospitalar Lisboa Norte - Hospital Santa Maria - Centro académico de Lisboa, Portugal

Background/Objective: Spondylodiscitis is a rare entity in pediatrics. The low specificity of symptoms and laboratory data, the scarcity of physical examination findings and the late radiographic changes justify the usually late diagnosis.

Methods: 15-month-old boy with irrelevant personal and family history. Presented to our emergency department with notion of pain in the previous 15 days, characterized by periods of polypnea and irritability. Progressive worsening in the previous week, with nocturnal awakening, improvement with ibuprofen, and antalgic position in dorsal decubitus or lumbar hyperextension, with gait refusal in the last days. History of diarrhea 2 weeks before admission. He looked prostrate, complaining while sitting but had no pain in passive mobilization of the lower limbs or in palpation/percussion of the spine. He started fever at the hospital that persisted for 2 days. The blood panel showed ESR 55mm and ALP 1076 U/L and no increase in CRP or leukocytosis. CT showed vertebral irregularity of D9-D10 with possible relation to bone destruction secondary to spondylodiscitis.

He was transferred to a tertiary hospital, where the MRI confirmed the diagnosis.

Serologies for Brucella, Bartonella and Coxiella, blood culture, IGRA and PCR for bacterial DNA were negative. After 8 days of intravenous ceftriaxone IV he was asymptomatic and was discharged with oral amoxicillin and clavulanic acid for 5 weeks.

Conclusion: Spondylodiscitis usually has an insidious onset of symptoms. Although children may report low back and walking pain, infants may initially only show nonspecific signs such as irritability. Therefore, it is important to remember this illness, especially as it may have serious complications. MRI is the key standard for an early diagnosis and follow-up of disease progression.

805

Infectious Diseases

Review of CNS Abscess in Children

Jose Andrade Guerrero ¹ , Serafín Castellano Dámaso ¹ , Marta Bautista Barea ¹ , Ines de Augusto Claudino ² , Mariano Silva Hernández ¹ , Virginia Santana Rojo ¹ , Marta Dorado Criado ¹ , Celia Fabra Garrido ¹ , Fernando Carceller Benito ³ , Javier Saceda Gutiérrez ³ , María Laplaza González ⁴ , Jesus Domínguez Riscart ¹ , Carmen Román Hernández ⁴ , Miguel del Rio Garcia ⁴ , Marta García-Fernández de Villalta ¹ , Francisco Climent Alcalá ¹ , Ana Méndez Echevarría ¹ , Luis Alonso García ¹ , Cristina Ots Ruiz ¹ , Fernando Baquero-Artigao ¹ , María Fátima Ara Montojo ¹ , Luis Escosa Garcia ¹ , Francisco Javier Aracil Santos ¹

¹ General Pediatrics and Infectious Diseases, Hospital Universitario La Paz, Spain

² General Pediatrics and Infectious Diseases, Centro Hospitalar Oeste, Portugal

³ Pediatric Neurosurgery, Hospital Universitario La Paz, Spain

⁴ Pediatric Intensive Care Unit, Hospital Universitario La Paz, Spain

Background: CNS abscesses are rare and severe conditions. These infections are categorized based on their location: epidural abscess, subdural empyema and brain abscess. Treatment requires a combination approach with antibiotic therapy and frequently surgical drainage.

Objective: To describe the clinical manifestations, predisposing factors, diagnosis, treatment and outcome of pediatric patients with CNS abscesses

Methods: Retrospective single-center study of pediatric patients with CNS abscesses in the 2000-2018 period. Neonatology patients were excluded.

Results: A total of 32 patients were included in the study (brain abscess 31%, subdural empyema 31%, brain + subdural 6%, epidural abscess 25%). Age range was 1,5 months to 16 years (median 6 years). Male/female ratio was 2:1. Predisposing factors were identified in all patients. Most important predisposing factors were mastoiditis and sinusitis (56%), complex congenital cardiopathy (16%) and bacterial meningitis (13%). Etiologic agents were identified in 18 patients (Staphylococcus aureus 28%; Streptococcus pneumoniae 28%; Streptococcus intermedius 22%; gram-negative bacilli 17%). Fever, headache, earache, vomits and seizures (41%) were the signs and symptoms most often described. Half of patients underwent surgery of the CNS abscess (brain abscess 75%, epidural abscess 25%). Two main initial antibiotic schemes were used, cefotaxima-vancomycin-metronidazole (42%) and meropenem-vancomycin (48%). More frequently used oral antibiotics were quinolones, metronidazole and linezolid. Median of duration of antibiotic treatment (intravenous + oral) was 69 days (range 40-120). One patient died and seven had permanent sequels.

Conclusions: Congenital heart diseases and otorhinolaryngological infections were the main underlying disorders. Antibiotic therapy was enough to resolve small epidural abscesses and small subdural empyema. But most patients with brain abscesses and big subdural empyema also required surgical treatment.

815

Infectious Diseases

Drug Related Adverse Events During Treatment of CNS Abscess in Children

Marta Bautista Barea ¹ , Serafín Castellano Dámaso ¹ , Jose Andrade Guerrero ¹ , Ines de Augusto Claudino ² , Mariano Silva Hernández ¹ , Virginia Santana Rojo ¹ , Marta Dorado Criado ¹ , Celia Fabra Garrido ¹ , Fernando Carceller Benito ³ , Javier Saceda Gutiérrez ³ , María Laplaza González ⁴ , Jesus Domínguez Riscart ¹ , Miguel del Rio Garcia ⁴ , Carmen Román Hernández ⁴ , Marta García-Fernández de Villalta ¹ , Francisco Climent Alcalá ¹ , Ana Méndez Echevarría ¹ , Luis Alonso García ¹ , Cristina Ots Ruiz ¹ , Fernando Baquero-Artigao ¹ , Luis Escosa Garcia ¹ , María Fátima Ara Montojo ¹ , Francisco Javier Aracil Santos ¹

¹ General Pediatrics and Infectious Diseases, Hospital Universitario La Paz, Spain

² General Pediatrics and Infectious Diseases, Centro Hospitalar Oeste, Portugal

³ Pediatric Neurosurgery, Hospital Universitario La Paz, Spain

⁴ Pediatric Intensive Care Unit, Hospital Universitario La Paz, Spain

Background: Treatment of brain abscesses and other intracranial infections requires long lasting antibiotics, glucocorticoids and antiepileptic therapy. Antibiotics with good diffusion to CNS and inside abscesses are the preferred ones. Medicaments related adverse events are frequent in these patients. Generally, they force to change the planned treatment.

Objective: To describe drugs related adverse events during treatment of pediatric patients with CNS abscesses.

Methods: Retrospective single-center study of pediatric patients treated for CNS abscesses.

Results: A total of 32 patients were included in the study (2000-2018). Presented with brain abscess 31%, subdural empyema 31%, brain abscess + subdural empyema 6% and epidural abscess 25%. Age range was 1,5 months to 16 years (median 6 years). Male/female ratio was 2:1.

Usually, these patients received sequential intravenous and oral treatment. Median of duration of antibiotic treatment (intravenous + oral) was 69 days (range 40-120). Intravenous treatment median was 28 days (range 7-90 days). Received glucocorticoids 47% and antiepileptic drugs 62%.

Three patients developed neutropenia (related to linezolid and cotrimoxazole). Four patients developed exanthems. Three patients developed DRESS syndrome (one due to phenytoin and two related to antibiotics). Two patients had knee arthritis related to fluoroquinolones. One patient developed linezolid related peripheric neuropathy. Cushing syndrome was related to prolonged glucocorticoid therapy.

Conclusions: Patients treated for CNS pyogenic infections frequently develop drug related adverse events. Careful monitoring of blood counts, liver and renal function and skin disorders is mandatory.

435

Infectious Diseases

Intelligent IT System Developed to Reduce the Risk of TORCH Congenital Infections

Andreia Florina Nita ^1,2,3 , Loredana Sabina Cornelia Manolescu ^1,2 , Gabriela Loredana Popa ^1,2,4 , Mircea Ioan Popa ^1,2

¹ Microbiology, Carol Davila University of medicine and Pharmacy, Romania

² Microbiology, Cantacuzino National Medico-Military Institute for Research and Development, Romania

³ Paediatrics, Grigore Alexandrescu Emergency Hospital for Children, Romania

⁴ Parasitology, Colentina Clinical Hospital, Romania

Introduction: TORCH congenital infections have a substantial burden of disease, leading to non-genetic, preventable long-term sequelae. Effective interventions for prevention of mother-to-child transmission of TORCH infections are not implemented in Romania. Furthermore, there is a lack of national protocols and guidelines regarding screening and diagnosis of TORCH congenital infections.

Methods: The current project was carried out as a collaboration between medical experts from Carol Davila University of Medicine and Pharmacy and an information technology team from Stefan S Nicolau National Institute of Virology. The project was supported from UEFISCDI Bridge Grant: PN-III-P2.1-BG-2016-0384 and performed during 2016-2018.

Results: We have developed an intelligent system for pregnant women and medical staff, gathering information about the infections tests in TORCH panel and enclosing algorithms for correct screening and diagnosis of TORCH infections in order to reduce the risk of congenital infections. Using practical and non-invasive electronic resources widely available such as smartphones, the authors have created a website portal (https://www.torch.ro/) and an application for mobile phones (MAMI) which allows women to have quick access to correct information about TORCH infections and tests and more important, to obtain an individualized answer regarding TORCH screening and diagnosis which can contribute to further planning and case management carried out by the physician.

Conclusion: TORCH-IT portal and MAMI app for smartphone, developed to reduce the risk of congenital infections while helping to obtain a correct diagnosis and management, are expected to become valuable resources for both the patient and the physician, as they are unique in Romania.

161

Infectious Diseases

Scalp Cellulitis of Unexpected Cause

Ruxandra Simona Bacanu ¹ , Vianney Marienela Cuba Mamani ²

¹ Pediatrics, General Hospital Tomelloso, Spain

² Emergency Department, General Hospital Tomelloso, Spain

Background: Cellulitis is usually caused by a bacterial infection of an area of skin that is no longer intact. Other causes may include human or animal bites, or injuries that occur in water.

Aim of Paper: To demonstrate that there are weird causes that lead to a cellulitis and the importance of a exhaustive differential diagnosis.

Matherial and Method: We present the case of a 5 years old girl who arrived to Emergency Room with scalp cellulitis. Six days before, the parents noticed the presence of a wart in the girl´s scalp that during later days associated pain and redness in the area, accompanied by fever. The girl was attended in Primary Care and was in treatment with Azitromicin. The girl did not had history of trauma or other event.The physical exam revealed a healthy child with a painfull, red scalp area about 5 cm, covered by a crust.

We asked for urgent blood tests which revealed elevated acute phase reactants. It has been iniciated intravenous treatment with Augmentine. 2 days later, after scratching, expels a tick.

We asked for complete serology tests for Rickettsia and Borrelia, that luckily were negative. The microbiology test informed us about the infection of the bite area with Pantoea agglomerans.

The patient had a favorable evolution. After 2 months the ulcer has healed completely but with the persistence of a central scarring alopecia.

Disscusion: As we previous comment, there were no history of trauma or other event. The presence of a tick was a surprise for everybody (the tick was previously interpreted as a wart). The luck was that by scratching it fell into bed, and also that all the serology tests were negative.

Conclusion: We must always think of all the possible etiologies, no matter how weird it would seem to us.

615

Infectious Diseases

A Peculiar Scalp Lesion

Gabriela Botelho ¹ , Susana Gomes ¹

¹ Pediatria, Hospital do Espírito Santo de Évora, Portugal

Background: Tinea is a fungal infection and it is an increasing problem worldwide. Kerion is a severe manifestation of tinea capitis developing from an intense immune response to the infection, which is less common than the non-inflammatory variants. It can often mimic other conditions, leading to inaccurate diagnosis and delay in treatment with scarring alopecia.

Objective: Our goal is to report a case of atypical presentation of tinea capitis and highlight the importance of a careful clinical history and examination and timely diagnosis.

Methods: We analyzed the atypical presentation of a tinea capitis scalp infection and reviewed the literature.

Results: A three-year-old girl presented with a painful, soft plaque in the scalp, 3 cm of larger diameter, with thick crusting, pustules, dark spots, broken hair strands and some drainage areas, evolving for two weeks. She had no other skin lesions nor fever. There had been no trauma, contact with animals nor contacts with similar lesions. After inspection of the lesion, our hypothesis was a kerion. Empirical treatment with oral fluconazole and topic cetoconazol was begun. There were not any lasting lesions nor scarring, after four weeks of treatment.

Conclusion: Kerion is often misdiagnosed. Our choice of antibiotic was fluconazole and cetoconazol, as griseofulvin is not available in our country and terbinafine is not available as oral solution. Timely diagnosis and treatment led to recovery without scarring or residual alopecia.

811

Infectious Diseases

Evaluation of Antiviral Therapy for Influenza and Influenza-Like Diseases in Risk Groups

Vilya Bulgakova¹, Natalya Pshenichnaya², Artem Poromov³, Antonina Grekova⁴, Elena Kareva^5,6

¹ Institute of Pediatrics, National Medical Research Center of Children's Health, Russia

² Scientific Department, Central Research Institute of Epidemiology, Russia

³ Scientific Department, Lomonosov Moscow State University, Russia

⁴ Department of Infectious diseases, Smolensk State Medical University, Russia

⁵ Department of Pharmacology, Pirogov Russian National Research Medical University, Russia

⁶ Department of Pharmacology, I.M. Sechenov First Moscow State Medical University, Russia

Background: Bacterial complication and prolonged course of influenza and influenza-like illnesses (ILI) are often observed among patients with risk factors for severe course of disease. The aim of study was to evaluate the efficacy of antiviral therapy with arbidol (umifenovir) in hospitalized patients with influenza and ILI who had risk factors for severe course of disease.

Methods: 3285 medical charts of patients with influenza and ILI have been retrospectively reviewed. Laboratory confirmation of disease was available in 31.0% patients; of these, influenza viruses were found in 60.7% samples (influenza A/H1N1 - 48.8%, A/H3N2 - 16.3%, influenza A subtype–10.6%, influenza B - in 24.3%, other respiratory viruses - 39.7%). Patients were divided into 2 groups depending on therapy. Antivirals were not administered to patients in the 1th group. 2th group of patients was treated with umifenovir from the first 24-48 hours after symptom onset. Patients in subgroups 1b (n = 155) and 2b (n = 628) had risk factors (children younger than 2 years old and adults over 65, pregnant women, people with chronic somatic diseases and obesity).

Results: Duration of fever and frequency of complications in patients without risk factors were significantly lower in 2a group versus 1a group: 2.86 ± 1.57 days and 3.76 ± 2.83 days, 13.1 ± 1.3% and 20.0 ± 3.2%, accordingly. Duration of fever and frequency of complications in patients with risk factors were significantly lower in 2b group versus 1b group: 3.0 ± 1.48 days and 4.1 ± 2.2 days, 9.9 ± 1.2% and 42.6 ± 3.6%, accordingly.

Conclusion: The efficacy of antiviral therapy was higher in patients with risk factors than in patients who are not at risk. In patients treated with umifenovir the duration of fever and frequency of complications were lower than in patients who did not receive antiviral therapy.

826

Infectious Diseases

Characteristics of Influenza and Influenza-Like Diseases in Risk Groups

Vilya Bulgakova ¹ , Natalya Pshenichnaya ² , Artem Poromov ³ , Antonina Grekova ⁴ , Elena Kareva ^5,6 , Elena Obraztsova ⁷

¹ Institute of Pediatrics, National Medical Research Center of Children's Health, Russia

² Scientific Department, Central Research Institute of Epidemiology, Russia

³ Scientific Department, Lomonosov Moscow State University, Russia

⁴ Department of Pediatrics, Smolensk State Medical University, Russia

⁵ Department of Pharmacology, Pirogov Russian National Research Medical University, Russia

⁶ Department of Pharmacology, I.M. Sechenov First Moscow State Medical University, Russia

⁷ Department of Pediatrics, Research Institute of Influenza, Russia

Aim: To identify risk factors (RFs) for the development of bacterial complications and the prolonged course of influenza and other acute respiratory viral infections (ARVIs) among inpatients treated in Russian healthcare facilities in the post-pandemic period; to determine the clinical presentation of the disease (flu-like syndrome) in risk-group people and to evaluate the efficacy of antiviral therapy with arbidol (umifenovir).

Methods: The investigators retrospectively analyzed randomly selected medical records of inpatients with influenza and other ARVI in 88 hospitals from 50 regions of the Russian Federation: those of 5287 patients, respectively, by applying parametric and nonparametric statistical methods.

Results: Single-factor analysis of variance revealed factors influencing the course of flu-like syndrome and identified risk groups: children younger than 3 years old and adults over 65, pregnant women, and people with chronic somatic diseases and obesity. The high-risk groups exhibited a more severe course of flu-like syndrome than did the patients outside the risk groups. The incidence of complications was higher, especially in the under 2-year-year-old children and in patients with endocrine, metabolic, or respiratory diseases, with a large proportion of complications being pneumonia. The efficacy of antiviral therapy (umifenovir) was higher in patients with chronic diseases than in patients not at risk. The duration of fever and frequency of complications proved to be lower than those in patients who did not receive antiviral therapy.

Conclusion:The RFs for influenza and ARVI complications are patient`s age (children under 3 years of age and adults older than 65 years), the presence of chronic somatic diseases, and pregnancy. Patients with endocrine, eating, metabolic (including obesity), circulatory, and respiratory disorders are at high risk for influenza and ARVI complications. Umifenovir therapy substantially reduces the duration of fever and risk of complications, especially in patients with laboratory-confirmed influenza infection.

259

Infectious Diseases

Incomplete Kawasaki Disease on a 4 Month Old Child: When Fever is the only Clinical Sign

Bernardo Camacho ¹ , Alexandra Andrade ¹ , Carolina Freitas Fernandes ¹ , Carolina Ferreira Gonçalves ¹ , Joana Oliveira ¹ , Cristina Freitas ¹ , Conceição Freitas ¹

¹ Department of Pediatrics, Hospital Central do Funchal, Portugal

Background: Kawasaki disease (KD) is one of the most common vasculitis of childhood and remains the main cause of acquired heart disease in children in developed countries. It most commonly (90%) affects children before 5 years of age and less frequently (10%) before 6 months of age. The pathophysiology is still unknown, but immunologic factors appear to play an important role.

Objective: We present a case that highlights the importance of clinical suspicion of KD, even in the small infant.

Methods – case report: A four-month-old female infant was admitted for fever and irritability for 5 days. Vaccination with Meningococcal Group B Vaccine had been instituted 2 days before the show of symptoms. On admission, there were no remarkable physical findings. Laboratory evaluation showed elevated white blood cell count (WBC) and C Reactive Protein (CRP), urinalysis was normal. Blood and urine cultures were obtained and intravenous Ceftriaxone was started. On the 4th day of admission the patient was afebrile, but because of maintained irritability and tympanic temperatures constantly above 37.5 °C, laboratory evaluation was repeated. WBC and CRP values remained elevated, now with associated thrombocytosis and elevated liver enzyme values. Cultures showed no bacterial growth. On the 8^th day, due to the maintained clinical status, laboratory evaluation was once again repeated, and continued to show a rise of the inflammatory markers. KD was considered and echocardiographic evaluation was performed, showing diffuse ectasia of the right coronary arteries (3mm). Treatment with IV immunoglobulin and acetylsalicylic acid was started, with clinical improvement.

Conclusion: KD remains an infrequent illness before 4 months of age and its aetiology a cause of debate. This case report highlights the importance of clinical suspicion of KD, even when fever is the only positive diagnostic criteria.

549

Infectious Diseases

Pediatric Haemophilus Infuenza Invasive Disease in a Portuguese Second-Level Hospital: 2010-2018

Mafalda Casinhas Santos ¹ , Sara Limão ¹ , Florbela Cunha ¹

¹ Pediatric Service, Hospital Vila Franca de Xira, Portugal

Introduction:Haemophilus influenza is a nasopharynx colonizer in children, responsible for upper respiratory tract infections and rarely causes invasive disease, namely meningitis, pneumonia, arthritis or bacteremia. Before routine immunization against Haemophilus influenza type b (Hib) was included in National Vaccination Program (NVP) in 1994, this was the main responsible for invasive disease. Afterwards, non-capsulated (NC) strains have been the most frequently implicated.

Methods: Retrospective observational study describing all pediatric cases of invasive Haemophilus influenza disease in a second-level hospital between 2010 and 2018.

Results: Seven cases were identified: three Hib and four NC strains. There were five boys and two girls and a mean age was 18 months (3 m -11 y).

Hib vaccination was complete in all children except for one, who wasn`t vaccinated. Major risk factors were prematurity (2), asthma (1), psychomotor development retardation (1), recurrent acute otitis (1), recent tonsillectomy (1) and IgA deficiency (2). Two vaccine failures occurred, a girl with a recent tonsillectomy and a boy with IgA deficiency.

Diagnosis was confirmed by isolation of Haemophilus from blood (6), cerebrospinal fluid (3) and pleural fluid (1). Clinical presentations were meningitis (4), three of them with septicemia, pneumonia (2) and occult bacteremia (1). Of the Hib infection cases, two presented with meningitis and one with occult bacteremia. One patient with Hib meningitis developed permanent neurosensorial hear loss and ataxia. Three patients were admitted to Intensive Care Unit for a medium of four days.

Third generation cephalosporins (5) and ampicillin (2) were the chosen antibiotics. Ampicillin resistance mediated by beta-lactamases was identified in one patient.

Discussion:Haemophilus influenza invasive disease represents a potential serious condition. Although NC strains are the most prevalent, Hib is still present and responsible for some cases

National epidemiological surveillance is of crucial importance and may help the development of new preventive strategies.

1033

Infectious Diseases

Risk Factors Associated with Bacteremia Due to Multi-Drug Resistant Pseudomonas Aeruginosa in a Pediatric Tertiary Referral Hospital in Mexico City

Adrian Ceballos Bocanegra ¹ , Gabriela Guzman Gutierrez ³ , Martha Aviles Robles ²

¹ Pediatrics, Centro Medico Dalinde, Mexico

² Infectious Diseases, Hospital Infantil de México Federico Gómez, Mexico

³ Pediatrics, Hospital Infantil de México Federico Gómez, Mexico

Background: The incidence of multi-drug resistant P. aeruginosa (MDR-PA) has increased in recent years. There is an association between antimicrobial resistance and the increased in mortality and prolonged hospitalization.

Objective: To identify risk factors associated with the acquisition of bacteremia due to MDR-PA in pediatric patients

Methods: We performed a retrospective case -control study from 2012 to 2018 in Hospital Infantil de México Federico Gómez in Mexico City. Cases: patients with positive cultures for MDR-PA. Controls: patients with cultures positive for P. aeruginosa susceptible to at least 3 groups of antibiotics. To assess for possible risk factors we perform crosstabs and calculation of odds ratio (OR). A multiple logistic regression model was conducted with significant variables.

Results: We analyzed 130 subject (46 cases and 84 controls). Mean age 6.6 years. The Most frequent basal condition was oncologic (42%) and the most frequent diagnosis septic shock (53%). Cephalosporins (56%) was the previous antibiotic more frequently used before the event of bacteremia by MDR-PA. Meropenem was the antibiotic most used as empirical treatment (58.4%).: Previous use of broad spectrum antibiotics (OR 8.2, 95% CI 1.86 - 36.65, p 0.01) and previous stay in PICU (OR 4.9, 95% CI 1.21- 20.39, p 0.01) were associated to bacteremia due to MDR-PA.. There were more cases of complication in the group of MDR-PA bacteremia: septic shock (97.8%, P 0.01) and admission to UTIP (63.3%

Conclusion: In our population, risk factors for acquisition of bacteremia due to MDR-PA were previous use of antibiotics and previous stay in PICU. Mortality from bacteremia due to MDR-PA was high (51.8%).

1085

Infectious Diseases

Indications for and Outcomes of Lumbar Punctures Performed in Infants less than 3 Months Old

E. Clyde ¹ , J. Orr ¹ , S. Alexander ¹

¹ Royal Hospital for Sick Children, UK

Background: Infants under 3 months often present with nonspecific signs and symptoms making it a challenge to diagnose CNS infection in this population. Yet missing the diagnosis can result in significant mortality and morbidity. A recent large review showed low rates of bacterial meningitis (BM) in the under two month population, contributing to increased interest in whether low risk infants can be identified to reduce the need for invasive investigations.

Objectives: To look at indications for and results of LP in the less than 3 months population presenting to our hospital and determine whether features exist to aid in stratifying infants as low risk of BM.

Methods: Data was retrospectively collected on all infants under 3 months who had LP performed between September 2017 and September 2018. Using the hospital's electronic database we gathered demographic data, initial observations, presenting features and documented indication for LP alongside results from biochemical and bacteriological investigations.

Results: 134 LP were performed, 43 in the less than 1 month group and 91 in the 1-3 months group. There were no positive bacteriology results with 26 (19.4%) confirmed cases of viral meningitis. 3 (0.02%) patients were treated for presumed BM (1 was clinically suspected with no LP performed, 1 had raised WCC with positive CSF microscopy and 1 was found to have meningeal thickening on cranial ultrasound scan). See chart 1 for summary of diagnosis at time of discharge.

Chart 1: Diagnosis at the time of discharge for all infants under 3 months of age on whom an LP was performed between September 2017 and 2018

Conclusion: This data adds to a growing body of evidence of low rates of positive results from LP in the under 3 month group. Further research is required to better stratify this population and avoid unnecessary invasive investigation without missing the diagnosis in the small proportion of infants who do have BM possibly allowing a more individualised approach to febrile under 3 month old patients.

448

Infectious Diseases

Acute Osteomyelitis: A Retrospective Study

Sofia Cochito Sousa ¹ , Patrícia Lipari Pinto ¹ , Paula Correia ¹ , Teresa Ferreira ¹ , Pedro Beckert ² , Helena Cristina Loureiro ¹

¹ Departamento da Criança e Jovem, Hospital Prof. Doutor Fernando Fonseca, EPE, Portugal

² Serviço de Ortopedia, Hospital Prof. Doutor Fernando Fonseca, EPE, Portugal

Background: Acute osteomyelitis(AO) is a rare but serious infection in children, as it can cause significant morbidity.

Objective: Characterization of children with AO in a suburban area outside Lisbon.

Methods: Retrospective study of children (18years) hospitalized for AO between 2012 and 2018. Record data: demographic, clinical, laboratory, imagiologic and therapeutic information. Statistical analysis performed with IBM-SPSS®.

Results: 40 children were included, the majority male (60%) and of African origin (63%) with mean age of 7.9±5.4years.

Lower limb was the most affected (75%), with predominance of femur (35%). Concomitant septic arthritis was present in 28%, being coxofemoral the most affected joint.

Main symptoms at presentation were pain at mobilization (90%), spontaneous pain (85%), fever (60%) and local inflammatory signs (55%), with median duration before admission of 2.0days (IQR 1.0-6.0). 45% had leukocytosis, 3% leukopenia, 79% elevated erythrocyte sedimentation rate(20mm/h) and 58% elevated C-reactive protein(2mg/dL).

53% had risk factors: sickle-cell disease (43%), acute mastoiditis (5%), smallpox (2.5%) and preceding fracture (2.5%).

Agent identified in 18 (45%): S. aureus 7 (MSSA 5, MRSA 2), Streptococcus pneumoniae 3, Escherichia coli 3, Streptococcus pyogenes 2, Neisseria meningitidis 1, Mycobacterium bovis 1, Candida famata 1. Methods: culture media (89%) and detection of bacterial DNA by PCR (11%).

Magnetic resonance(MRI) was performed in 95%. In all, signs of OA were present.

All were treated with antibiotics with median duration of 6.1weeks (IQR 6.0-8.6). Mycobacterium bovis infection was treated with anti-tuberculosis drugs and fungal infection with antibiotics and antifungal drugs. 35% needed surgery and 8% hyperbaric oxygen therapy.

There were 11 complications: subperiosteal abscess (n=6;15%), deep soft tissue abscess (n=1;3%), venous thrombosis (n=4;10%).

Conclusion: We found a high prevalence of african children and sickle-cell disease. In a child with fever and bone pain, especially if elevation of acute phase reactants, we should consider OA and perform a MRI.

478

Infectious Diseases

Guillain-Barré Syndrome in a Level II Hospital: Pre and Post Protocol

Sara Completo ¹ , Marta Veríssimo ¹ , Carlos Escobar ¹¹ , Paula Correia, Catarina Luís ¹

¹ Pediatrics Department, Hospital Prof. Doutor Fernando Fonseca, E.P.E., Portugal

Background: Guillain-Barré Syndrome (GBS) is a heterogeneous group of acute immune-mediated polyneuropathies. Incidence is lower in children than in adults and there is usually complete recovery. In 2011 the Portuguese Neuropediatric Society issued a diagnosis and management protocol for GBS that allows a more systematic orientation of patients and earlier treatment (when criteria are met), avoiding possible complications.

Aim: Characterize GBS pediatric patients in a level II hospital; compare the approach before and after the protocol.

Methods: Retrospective analysis of the epidemiological, laboratory, clinical, treatment and follow-up data of the cases admitted to our hospital with GBS between 2011-2018 (second group – post-protocol), and comparison with a previous study (2010) which included patients from 1997 to 2009 (first group – pre-protocol). Data was analyzed with Excel.

Results: The first study group had six patients, medium age 4,5 years, and the second, four patients, medium age 4,3 (total: seven masculine, three feminine). All had lower extremity weakness and areflexia. Other symptoms included: pain, paresthesia, cranial nerve involvement, and fever. Eight progressed to superior extremity weakness, and three had dysautonomic disfunction. One required mechanical respiratory support.

Most (9/10) had previous infection. All patients underwent lumbar puncture (LP), 8/10 had albuminocytologic dissociation. 9/10 had electromyography (EMG) compatible with peripheral polyneuropathy. Pre-protocol, 5/6 were treated with Immunoglobulin (Ig) and 2/6 with corticosteroids; Post-protocol, all patients were treated with Ig; Two repeated Ig, due to clinical worsening. 9/10 had complete recovery and one abandoned the follow-up, with partial areflexia after three months.

Conclusion: According to the protocol, corticosteroids are contraindicated and weren’t used on any patient from the second group. With atypical symptoms, LP and EMG can be helpful. Almost all patients had total recovery of the symptoms, which is frequent in children. The protocol permits a more uniformized approach to GBS.

226

Infectious Diseases

Cervical Lymphadenopathies Refractory to Antibiotic Treatment

Ana Luisa Correia , Ana Lia Gonçalo, Ana Carolina Castro, Rui Almeida, M. Sofia Aroso

Pediatrics, Hospital Pedro Hispano, Portugal

Background: Cervical lymphadenopathies are a common occurrence among children. They are often associated with an infectious process and can be caused by multiple pathogens. Identifying an etiology can be challenging, as well as choosing the appropriate treatment.

Case Report 1: 11-month-old girl with a 2 days complaint of limitation of neck movement, without fever or other symptoms. She presented with voluminous and hardened bilateral cervical tumefaction despite having a good general appearance. Blood tests showed leukocytosis and a high C-reactive protein. Cervical ultrasound revealed unspecific features and the neck CT exhibited numerous reactive adenomegalies, the largest one with necrotic center. She started antibiotic therapy with ceftriaxone, clindamycin and azithromycin. Even so, she developed fever that lasted 10 days.

Case Report 2: 10-month-old girl with a 9 days history of left submandibular growing mass, with no history of fever or any other symptoms. Physical examination identified a palpable indurated left submandibular mass. Cervical ultrasound revealed findings consistent with bilateral adenitis. Blood work revealed leukocytosis and a high C-reactive protein. She started amoxicillin/clavulanic acid and azithromycin. She developed fever from day 1 until day 3.

Etiological investigation was negative for both patients, including EBV, CMV, Bartonella, Toxoplasmosis and tuberculosis, as well as blood cultures. After more than 15 days without clinical improvement, they underwent surgical drainage of the lymph node, and bacteriologic exam of pus identified a Staphylococcus aureus (sensitive for the antibiotics used empirically).

Conclusion:Staphylococcus aureus is one of the main pathogens implied on lymphadenitis. Generally, there is a sudden onset of inflammatory signs, with appearance of poorly mobile cervical lymphadenopathies. About 30% of the infected nodes suppurate within two weeks. With these cases we pretend to emphasize that treatment of Staphylococcus aureus lymphadenitis can be difficult, even when appropriate antibiotics are used resulting in a n eed for surgical drainage.

523

Infectious Diseases

Mastitis in Adolescents: Acknowledging New Risk Factors

Vanessa Costa ¹ , Raquel Alves ² , Filipa V Espada ²

¹ Paediatrics, Centro Hospitalar e Universitário do Porto, Portugal

² Paediatrics, Unidade Local de Saúde de Matosinhos - Hospital Pedro Hispano, Portugal

Introduction: Mastitis is uncommon in pediatrics and can be associated with breast abscess. In young nulliparous women there is a causal relationship with smoking habits, due to ductal and periductal inflammation. Disease severity and invasion is higher in the presence of risk factors such as cystic breast lesions. Clinical severity and treatment resistance results from poor penetration of antibiotics into breast tissue.

Case report: 17-year-old adolescent, nulliparous, menarche at age 12, with regular menses and not on oral contraceptive medication. Denied tobacco or other consumptions but exposed to second-hand smoke since early childhood. History of breast asymmetry since puberty, with an epidermoid cyst in her right breast diagnosed at 16 years of age. The patient presented acutely to the emergency department in March 2018, with painless heat and swelling of the right breast. Physical examination revealed a firm and well-defined tumefaction of the right breast, painful on palpation. No axillary lymph nodes were palpable. Breast ultrasound was performed due to the adolescent’s medical history and breast inflammatory signs, which revealed a 13mm epidermoid cyst with adjacent fat densification and fluid collection suggestive of mastitis. Started on oral flucloxacillin, the lesion presented a slow and gradual, however incomplete, reduction in size and inflammatory signs, with resolution only after 6 weeks of antibiotic treatment and surgical drainage.

Discussion: We present this case report as it is uncommon for a nulliparous adolescent to develop mastitis, thus considering the likely impact of second-hand smoke on its pathogenesis, as active smoking is considered a major risk factor for breast inflammatory lesions. In conclusion, we emphasize how tobacco exposure not only has a role on the development of comorbilities such as lung cancer and chronic pulmonary disease, but also other less frequent but equally severe pathological conditions in younger age groups, reinforcing smoke-free environments for children.

772

Infectious Diseases

Roseoloviruses In Patients With Neurological Disorders

Tamara Amvrosieva¹, Katerina Divakova², Elena Kishkurno^1,3, Alexandra Arinovich¹, Alexandr Kudin⁴

¹ Laboratory of Natural Reservoir Infections, The Republican Research and Practical Center for Epidemiology and Microbiology, Belarus

² Pediatric Infectious Diseases Department, Belarusian State Medical University, Belarus

³ Infectious Diseases Department, Belarusian Medical Academy of Post-Graduate Education, Belarus

⁴ Infectious Diseases Unit №5, City Children's Infectious Clinical Hospital, Belarus

Background: Recent studies indicate that HHV-6 and HHV-7 may trigger some neurological disorders.

Methods: We detected HHV-6 and HHV-7 in 121 samples of clinical material (serum, peripheral blood cells, urine, saliva/buccal swabs, and CSF) from 31 patients with the following clinical diagnoses: encephalitis/meningoencephalitis, febrile seizures, epilepsy, children with unspecified febrile fever, psychotic disorders, chronic fatigue syndrome.Qualitative detection of HHV-6 DNA was carried out by real-time PCR with a commercial kit, and HHV-7 primers and probe (Wada et al., 2009) were used for detection of HHV-7 DNA.

Results: HHV-6 DNA was found in 6.45% patients (2 of 31): in saliva, cells and urine (n=1), in cells and serum (n=1). HHV-7 DNA we detected in 12.9% patients (4 of 31): in CSF with blood (n=1), in saliva and urine (n=1), in cells and saliva (n=1), in cells and CSF (n=1). Both HHV-6 and HHV-7 were found in 25.8% patients (8 of 31): 3 of them had both viruses in saliva, 1 – in blood cells; in other 4 children roseoloviruses were detected in variants – HHV-6+HHV-7 in saliva and HHV-7 in blood cells, HHV-6 in saliva and HHV-7 in cells, HHV-6 in urine and HHV-6+HHV-7 in buccal swabs, HHV-6+HHV-7 in saliva and HHV-7 in CSF and urine.The quantity of HHV-6 in urine was

Conclusion: Roseoloviruses are often detected in the clinical material of patients with neurological disorders. This can’t indicate its etiopathogenic role, since persistent carriage of latent roseoloviruses occurs in healthy controls. This requires further research.

349

Infectious Diseases

Evaluation of Diagnostic Tests in Pediatric Brucellosis

Marta Dorado Criado ¹ , Ursino Barrios Machain ¹ , Lucía Vigara Rey ⁴ , María Penín Antón ⁴ , Sara Murias Loza ³ , Rosa María Alcobendas Rueda ³ , Agustín Remesal Camba ³ , María Cruz Vecilla Rivelles ⁴ , Francisco Javier Aracil Santos ²

¹ Pediatrics, Hospital Universitario La Paz, Spain

² Pediatric Infectious Diseases, Hospital Universitario La Paz, Spain

³ Pediatric Reumathology, Hospital Universitario La Paz, Spain

⁴ Pediatrics, Hospital Universitario Príncipe de Asturias, Spain

Introduction and Objectives: Brucellosis is a worldwide zoonosis which is currently in clear regression in Europe. In Spain, the incidence has been decreasing since 1990, although some human cases persist. The historical importance of this disease and the current lack of experience on its management, make us present this retrospective study.

The objective of our study is to assess the clinical validation of diagnostic tests performed in pediatric patients with brucellosis.

Methods: Retrospective study of 13 pediatric patients diagnosed from 1989 to 2018 (30 years), in two hospitals of Madrid province. The cases have been identified from the registries of Microbiology Services, hospital admissions and outpatient clinics.

Results: Cases were diagnosed from 1992 to 2003. All patients except of one, recognized consumption of not pasteurized milk products. Twelve out of thirteen patients presented arthralgias and / or arthritis; the most frequent locations were hip and sacroiliac joints. The median duration was 20 days for joint symptoms and 18 days for fever (median maximum temperature 38.7°C).

The diagnosis was initially carried out wtih Rose Bengal test in all patients (100% positive). Table 1 shows results of main diagnostic tests.

Blood culture and bone scintigraphy weren’t carried out in all patients. Blood culture was positive in 50% and bone scintigraphy showed pathological findings in 60% of tested patients. Rifampicin combined with cotrimoxazole or doxycicline were the adopted treatment in most patients. One patient fell on a relapse after the initial treatment.

Conclusions: The number of patients diagnosed from brucellosis was small. We have not diagnosed any case of pediatric brucellosis in the last 15 years. Serological tests were positive in all cases. Erythrocyte sedimentation rate and C reactive protein were near normal in most patients. Blood cultures and bone scintigraphy were useful diagnostic tools in half of the patients.

494

Infectious Diseases

Streptococcus Pyogenes: An Emerging Cause of Severe Pneumonia

Celia Fabra Garrido¹, Marta Dorado Criado¹, Paloma Troyano Hernáez², Virginia Santana Rojo¹, Serafín Castellano Dámaso¹, Jose David Andrade Guerrero¹, Ursino Barrios Machain¹, Mariano Silva Hernández¹, Marta Bautista Barea¹, Adela García Perea², Iker Falces Romero², Teresa Del Rosal Rabes³, Fernando Baquero Artigao³, Luis Escosa García³, Cristina Ots Ruiz³, Talia Saínz Costa³, Clara Udaondo Gascón³, Luis Alfonso Alonso García³, Cristina Calvo Rey³, Ana Méndez Echevarría³, Ana Pérez⁴, Manuel Parrón Pajares⁴, María LaPlaza González⁵, Belén Calderón Iopis⁵, Jose Antonio Ruiz Dominguez⁶, Begoña De Miguel Lavisier⁶, Francisco Javier Aracil Santos³

¹ Pediatrics, Hospital Universitario La Paz, Spain

² Microbiology, Hospital Universitario La Paz, Spain

³ General Pediatrics and Infectious Diseases, Hospital Universitario La Paz, Spain

⁴ Radiology, Hospital Universitario La Paz, Spain

⁵ Pediatric Intensive Care Unit, Hospital Universitario La Paz, Spain

⁶ Pediatric Emergency, Hospital Universitario La Paz, Spain

Background and Aims:Streptococcus pyogenes is an uncommon cause of community-acquired pneumonia(CAP) in children, but it seems to have increased in the last years. It is associated with severe disease, including longer duration of fever and hospital stay, development of complications (nectrotizing pneumonia, bacteremia, toxic shock syndrome) and larger pleural effusions, with higher rates of bacterial isolation in pleural fluid, compared with other bacteria. The aim of this study is to review the clinical aspects of S. pyogenes-CAP diagnosed in in our hospital during the last two decades.

Methods: Retrospective descriptive study of pediatric inpatients with S. pyogenes-CAP in the period 2000-2019.

Results: We included 21 patients (16 boys and 5 girls), median age [interquartile range–IQR] at diagnosis was 25 [20-43] months. Yearly distribution of cases is shown in Figure 1. Median hospital stay [IQR] was 22 [11-26] days. A total of 17 patients (80%) were admitted to the Intensive Care Unit (median [IQR] stay 4 [2-8] days). All patients but two developed pleural effusion that required chest tube insertion. Sixteen patients (76%) required respiratory support. Pleural fluid culture was positive in 17/18 (94%). Blood cultures were positive in 6/20 (30%). Twelve patients out of 21 (57%) had positive viral detections in nasopharyngeal aspirates (identified by multiplex PCR), being influenza the most frequent one (5 cases). Regarding complications, 8 patients (38%) developed necrotizing pneumonia, 9 (43%) had hemodynamic instability and 5 (23%) toxic shock syndrome. All patients received intravenous antibiotics, followed by oral antibiotics with complete resolution.

Conclusions: We have observed an increase in the number of cases of S. pyogenes-CAP in the last years. Most patients were preschoolers with severe pneumonia and pleural effusion. These infections require prolonged hospital stay and frequent Intensive Care Unit admission, as complications occur often. However, all patients achieved a full recovery.

929

Infectious Diseases

Tuberculosis Meningitis: A Case Report

Carolina Figueiredo ¹ , Ana Teresa Raposo ¹ , Juan Gonçalves ¹ , M. Rita Soares ¹ , Fernanda Gomes ¹

¹ Pediatric Service, Hospital Divino Espirito Santo, Portugal

Background: Currently, Portugal presents low risk of Mycobacterium tuberculosis infection. In 2014, the incidence of the disease reached the limit of low incidence (20/100,000 inhabitants) that remains until the present time. In 2016, BCG vaccination strategy was changed from a universal strategy to selective (risk groups).

Objective: Case report of a 17-year-old adolescent with iron deficiency anaemia, who had been medicated for 3 months with oral iron, that went to urgency due to night-time holocranial headache that interfered with sleep associated with photophobia, phonophobia and night fever. He presented with anorexia, asthenia and weight loss of 8 Kg with 1 month of evolution. At admission, a decrease in the vesicular murmur in the right pulmonary base was identified. He did not present alterations in the neurological examination, namely meningeal signs. The blood count did not reveal changes and the inflammatory parameters were negative. A chest X-ray revealed small pleural effusion on the right. CT of the chest and molecular research of Mycobacterium tuberculosis in respiratory secretions were requested. He was hospitalized for an etiological study. An infectious context with risk contact was identified: the patient`s mother had a chronic cough with 1 year of evolution and a maternal uncle had recently been hospitalized for pulmonary tuberculosis. The patient under study would have been called for tuberculosis screening but did not attend. In day 5 of hospitalization, for maintenance of intense headache with little relapse to the therapy of relief, and appearance of stiffness of the neck, a lumbar puncture was performed that allowed identification of Mycobacterium tuberculosis complex by the DNA PCR technique.

Conclusion: Tuberculosis meningitis is a rare disease with a challenging diagnosis due to its insidious evolution. Targeted treatment should be initialized as soon as possible.

771

Infectious Diseases

Difficulties in the Diagnosis of Extrapulmonary Tuberculosis on a Refugee Teenager. A Case Report

Marie Bilger ¹ , Mathilde Arabska ¹ , Laurence Long ¹ , Houria Demil ¹ , Jean Stefaniuk ¹ , Stamatia Gkouliavoudi ¹

¹ Pediatric Departement, Sainte Catherine Hospital of Saverne, France

Background: Over recent years in France, the rate of tuberculosis remains stable with a declining tendency. 12.9% of tuberculosis cases concern teenagers and young adults from 15 to 24 years-old.

Objective: To reveal the importance of Polymerase chain reaction (PCR) in the diagnosis of bone tuberculosis.

Methods: A case report of a teenager with bone tuberculosis

Results: A 17-year-old Somalian boy without medical history was referred to our pediatric department with a 3-month history of fever, abdominal pain, vomiting, diarrhea, dysphagia to solids and serious anorexia. Refugee, his BCG vaccination status was unknown. He appeared unwell with dyspnea class II NYHA, bilateral inguinal and cervical painless lymphadenopathy. Pulmonary auscultation found decreased breath sounds on right lung. The C-reactive protein level was 57.4mg/L. The thoracic computed tomography (CT) found medium lobar consolidation with right-sided pleural nodules and bilateral inguinal lymphadenopathy. An abdominopelvic CT scan confirmed a 6-centimeter abscess in the spinal canal (T11-T12) and a 2-centimeter cystic lesion in the left iliac crest. QuantiFERON was positive. Neither a tuberculin purified protein derivative test nor a chest XR were performed. Expectorated sputum and gastric catheterization showed no sign of Mycobacterium tuberculosis (MT). First bone biopsy under local anesthesia failed due to pain and lack of patient cooperation. Meanwhile symptoms worsened. Any movement of the left hip became unbearable especially at night and while exercising. Lumbar spine and pelvic MRI revealed spondylitis involving T12, cold epidural and paravertebral abscess with compression sign and a cystic lesion of the right-sided sacrum. As the neurological exam was normal, no surgical decompression was recommended. A second biopsy was scheduled under general anesthesia. PCR confirmed MT, sensitive to rifampin. The patient regained normal function within a month.

Conclusion: PCR was very useful for the diagnosis of bone tuberculosis of a spinal lesion.

516

Infectious Diseases

Prepatellar Septic Bursitis in an 11 year old Girl

Joana Gonçalves ¹ , Ana Lança ¹ , Vivian Gonçalves ¹ , Paula Nunes ¹

¹ Department of Pediatrics, Centro Hospitalar Lisboa Ocidental, Portugal

Background: Prepatellar bursitis is an inflammation and fluid collection within the prepatellar bursa, typically due to trauma and less frequently related to infection, leading to septic bursitis. It is an extremely rare condition in the pediatric population.

Case Report: An 11-year-old obese girl presented to the emergency department reporting fever and an erythematous, swollen, and painful right knee following a trauma four days earlier. Two days before, she had been evaluated by an orthopedist who diagnosed a post-traumatic prepatellar bursitis. On admission she had normal vital signs, difficulty in gait and a knee with signs of inflammation and tender to palpation. There were insect bites in the skin overlying the area. Blood tests revealed: white cell count 13,6x10⁹/L, neutrophils 66,3%, sedimentation rate 52mm/h and C-reactive protein 17,2mg/dL. There were an increased volume of the soft tissue anterior to the patella at the x-ray. She was hospitalized and 24 hours later spontaneously drained purulent and serohematic content through a blister with 7 cm in diameter and the wound was cleaned. The ultrasonography showed a superficial abscess with a fistula to the exterior, with no intra-articular extension. The culture of bursal fluid revealed a methicillin-susceptible Staphylococcus aureus. She completed 7 days of endovenous flucloxacillin at the hospital and another 7 days orally at home. She had a favorable evolution, with no long-term complications.

Conclusion: If not treated correctly, septic bursitis can result in osteomyelitis with bone destruction. Early diagnosis with Gram staining and imaging is essential both to provide early adequate treatment and to exclude joint or bone involvement.

268

Infectious Diseases

Altered Consciousness Attributable to Unexpected Early-Onset Neurological Manifestation in Typhoid Fever: Case Report from Limited Resources Setting in East Nusa Tenggara, Indonesia

Angela Grace ¹ , Leonardus Wibowo Hidayat ¹

¹ General Medicine, St. Rafael General Hospital, Cancar, Indonesia

Background: Typhoid fever inflicts a significant burden of disease in endemic areas. While generally manifesting at a later course, multi-organ complications may occur prematurely. Typhoid delirium state, also known as typhoid encephalopathy or typhoid toxemia, is the most common and yet, under-recognized neurological complication.

Objective: To underline the challenges of diagnosing a severe typhoid fever with neurological complications in a limited resource setting.

Methods: A case of severe typhoid fever with early-onset neurological manifestations, initially suspected of acute psychosis, in a local low-resource private hospital in East Nusa Tenggara, Indonesia is presented. A 15-year-old undernourished boy presented with incoherent speech, generalized weakness, and disorganized behavior since the p