Skip to main content

Advertisement

SpringerLink
Log in

Childhood interstitial lung disease in Turkey: first data from the national registry

  • RESEARCH
  • Published:
European Journal of Pediatrics Aims and scope Submit manuscript

Abstract

The childhood interstitial lung diseases (chILD) Turkey registry (chILD-TR) was established in November 2021 to increase awareness of disease, and in collaboration with the centers to improve the diagnostic and treatment standards. Here, the first results of the chILD registry system were presented. In this prospective cohort study, data were collected using a data‐entry software system. The demographic characteristics, clinical, laboratory, radiologic findings, diagnoses, and treatment characteristics of the patients were evaluated. Clinical characteristics were compared between two main chILD groups ((A) diffuse parenchymal lung diseases (DPLD) disorders manifesting primarily in infancy [group1] and (B) DPLD disorders occurring at all ages [group 2]). There were 416 patients registered from 19 centers. Forty-six patients were excluded due to missing information. The median age of diagnosis of the patients was 6.05 (1.3–11.6) years. Across the study population (n = 370), 81 (21.8%) were in group 1, and 289 (78.1%) were in group 2. The median weight z-score was significantly lower in group 1 (− 2.0 [− 3.36 to − 0.81]) than in group 2 (− 0.80 [− 1.7 to 0.20]) (p < 0.001). When we compared the groups according to chest CT findings, ground-glass opacities were significantly more common in group 1, and nodular opacities, bronchiectasis, mosaic perfusion, and mediastinal lymphadenopathy were significantly more common in group 2. Out of the overall study population, 67.8% were undergoing some form of treatment. The use of oral steroids was significantly higher in group 2 than in group 1 (40.6% vs. 23.3%, respectively; p = 0.040).

  Conclusion: This study showed that national registry allowed to obtain information about the frequency, types, and treatment methods of chILD in Turkey and helped to see the difficulties in the diagnosis and management of these patients.

What is Known:

• Childhood interstitial lung diseases comprise many diverse entities which are challenging to diagnose and manage.

What is New:

• This study showed that national registry allowed to obtain information about the frequency, types and treatment methods of chILD in Turkey and helped to see the difficulties in the diagnosis and management of these patients. Also, our findings reveal that nutrition should be considered in all patients with chILD, especially in A-DPLD disorders manifesting primarily in infancy.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

Data availability

The data that support the findings of this study are available from the corresponding author upon reasonable request.

Abbreviations

BAL:

Bronchoalveolar lavage

ChILD:

Childhood interstitial lung diseases

ChILD-TR:

ChILD registry system in Turkey

CT:

Computed tomography

DLCOadj:

Adjusted diffusion capacity for carbon monoxide

DPLD:

Diffuse parenchymal lung disease

GERD:

Gastroesophageal reflux disease

FVC:

Forced vital capacity

FEV1:

Forced expiratory volume in 1 s

NEHI:

Neuroendocrine cell hyperplasia of infancy

TLC:

Total lung capacity

WLL:

Whole-lung lavage

References

  1. Nayir Buyuksahin H, Kiper N (2023) Childhood interstitial lung disease. Pediatr Allergy Immunol Pulmonol 36:5–15

    Article  PubMed  Google Scholar 

  2. Griese M (2022) Etiologic classification of diffuse parenchymal (interstitial) lung diseases. J Clin Med 11

  3. Cinel G, Kiper N, Orhan D, Emiralioglu N, Yalcin E, Dogru D, Ozcelik U, Oguz B, Haliloglu M (2020) Childhood diffuse parenchymal lung diseases: we need a new classification. Clin Respir J 14:102–108

    Article  PubMed  Google Scholar 

  4. Rice A, Tran-Dang MA, Bush A, Nicholson AG (2013) Diffuse lung disease in infancy and childhood: expanding the chILD classification. Histopathology 63:743–755

    Article  PubMed  Google Scholar 

  5. Kurland G, Deterding RR, Hagood JS, Young LR, Brody AS, Castile RG, Dell S, Fan LL, Hamvas A, Hilman BC, Langston C, Nogee LM, Redding GJ (2013) An official American Thoracic Society clinical practice guideline: classification, evaluation, and management of childhood interstitial lung disease in infancy. Am J Respir Crit Care Med 188:376–394

    Article  PubMed  PubMed Central  Google Scholar 

  6. Clement A, Nathan N, Epaud R, Fauroux B, Corvol H (2010) Interstitial lung diseases in children. Orphanet J Rare Dis 5:22

    Article  PubMed  PubMed Central  Google Scholar 

  7. Ferraro VA, Zanconato S, Zamunaro A, Carraro S (2020) Children's interstitial and diffuse lung diseases (ChILD) in 2020. Children (Basel) 7

  8. (2014) AHRQ Methods for Effective Health Care. In: Gliklich RE, Dreyer NA, Leavy MB (eds) Registries for evaluating patient outcomes: a user's guide. Agency for Healthcare Research and Quality (US), Rockville (MD)

  9. Griese M, Seidl E, Hengst M, Reu S, Rock H, Anthony G, Kiper N et al (2018) International management platform for children’s interstitial lung disease (chILD-EU). Thorax 73:231–239

    Article  PubMed  Google Scholar 

  10. Bush A, Cunningham S, de Blic J, Barbato A, Clement A, Epaud R, Hengst M, Kiper N, Nicholson AG, Wetzke M, Snijders D, Schwerk N, Griese M (2015) European protocols for the diagnosis and initial treatment of interstitial lung disease in children. Thorax 70:1078–1084

    Article  PubMed  Google Scholar 

  11. Clement A, de Blic J, Epaud R, Galeron L, Nathan N, Hadchouel A, Barbato A, Snijders D, Kiper N, Cunningham S, Griese M, Bush A, Schwerk N (2016) Management of children with interstitial lung diseases: the difficult issue of acute exacerbations. Eur Respir J 48:1559–1563

    Article  PubMed  Google Scholar 

  12. Seidl E, Schwerk N, Carlens J, Wetzke M, Cunningham S, Emiralioğlu N, Kiper N, Lange J, Krenke K, Ullmann N, Krikovszky D, Maqhuzu P, Griese CA, Schwarzkopf L, Griese M (2022) Healthcare resource utilisation and medical costs for children with interstitial lung diseases (chILD) in Europe. Thorax 77:781–789

    Article  PubMed  Google Scholar 

  13. Griese M, Kappler M, Stehling F, Schulze J, Baden W, Koerner-Rettberg C, Carlens J, Prenzel F, Nährlich L, Thalmeier A, Sebah D, Kronfeld K, Rock H, Ruckes C, Wetzke M, Seidl E, Schwerk N (2022) Randomized controlled phase 2 trial of hydroxychloroquine in childhood interstitial lung disease. Orphanet J Rare Dis 17:289

    Article  PubMed  PubMed Central  Google Scholar 

  14. Casey AM, Deterding RR, Young LR, Fishman MP, Fiorino EK, Liptzin DR (2020) Overview of the ChILD Research Network: a roadmap for progress and success in defining rare diseases. Pediatr Pulmonol 55:1819–1827

    Article  PubMed  Google Scholar 

  15. McKnight L, Schultz A, Vidic N, Palmer EE, Jaffe A (2023) Learning to make a difference for chILD: value creation through network collaboration and team science. Pediatr Pulmonol

  16. Nevel RJ, Deutsch GH, Craven D, Deterding R, Fishman MP, Wambach JA, Casey A et al (2023) The US national registry for childhood interstitial and diffuse lung disease: report of study design and initial enrollment cohort. Pediatr Pulmonol

  17. Kiper N, Emiralioglu N. Nutrition booklet for families of patients with chILD. Available from: www.klinikum.uni-muenchen.de/Child-EU. [Last accessed: January 6, 2020]

  18. Comes A, Wong AW, Fisher JH, Morisset J, Johannson KA, Farrand E, Fell CD, Kolb M, Manganas H, Cox G, Gershon AS, Halayko AJ, Hambly N, Khalil N, Sadatsafavi M, Shapera S, To T, Wilcox PG, Collard HR, Ryerson CJ (2022) Association of BMI and change in weight with mortality in patients with fibrotic interstitial lung disease. Chest 161:1320–1329

    Article  PubMed  Google Scholar 

  19. Rinaldi S, Gilliland J, O’Connor C, Seabrook JA, Mura M, Madill J (2021) Fat-free mass index controlled for age and sex and malnutrition are predictors of survival in interstitial lung disease. Respiration 100:379–386

    Article  PubMed  Google Scholar 

  20. Gilbert C, Bush A, Cunningham S (2015) Childhood interstitial lung disease: family experiences. Pediatr Pulmonol 50:1301–1303

    Article  PubMed  Google Scholar 

  21. Stubbs KH, McMahon MXH, Popler J, Berry RC, Sharp WG (2022) Multidisciplinary feeding treatment for a patient with interstitial lung disease. Pediatr Pulmonol 57:3183–3185

    Article  PubMed  Google Scholar 

  22. Ghisa M, Marinelli C, Savarino V, Savarino E (2019) Idiopathic pulmonary fibrosis and GERD: links and risks. Ther Clin Risk Manag 15:1081–1093

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Dziekiewicz M, Marczak H, Banasiuk M, Aksionchyk M, Krenke K, Banaszkiewicz A (2023) Characteristics of gastroesophageal reflux disease in children with interstitial lung disease. Pediatr Pulmonol 58:171–177

    Article  PubMed  Google Scholar 

  24. Nathan N, Griese M, Michel K, Carlens J, Gilbert C, Emiralioglu N, Torrent-Vernetta A, Marczak H, Willemse B, Delestrain C, Epaud R (2023) Diagnostic workup of childhood interstitial lung disease. Eur Respir Rev 32

  25. Griese M, Schwerk N, Carlens J, Wetzke M, Emiralioğlu N, Kiper N, Lange J, Krenke K, Seidl E (2023) Minimal important difference in childhood interstitial lung diseases. Thorax 78:476–483

    Article  PubMed  Google Scholar 

  26. Ring AM, Carlens J, Bush A, Castillo-Corullón S, Fasola S, Gaboli MP, Griese M, Koucky V, La Grutta S, Lombardi E, Proesmans M, Schwerk N, Snijders D, Nielsen KG, Buchvald F (2020) Pulmonary function testing in children's interstitial lung disease. Eur Respir Rev 29

  27. Spagnolo P, Bush A (2016) Interstitial lung disease in children younger than 2 years. Pediatr 137

  28. Verma N, Altmayer S, Hochhegger B, Barros MC, Rajderkar D, Mohammed TL (2021) ChILD: a pictorial review of pulmonary imaging findings in childhood interstitial lung diseases. Curr Probl Diagn Radiol 50:95–103

    Article  PubMed  Google Scholar 

  29. Liang TI, Lee EY (2020) Interstitial lung diseases in children, adolescents, and young adults: different from infants and older adults. Radiol Clin North Am 58:487–502

    Article  PubMed  Google Scholar 

  30. Nayir Buyuksahin H, Basaran O, Balık Z, Bilginer Y, Ozen S, Dogru D (2023) Interstitial lung disease in autoinflammatory disease in childhood: a systematic review of the literature. Pediatr Pulmonol 58:367–373

    Article  PubMed  Google Scholar 

  31. Doğru D, Yalçin E, Aslan AT, Ocal T, Ozçelik U, Güçer S, Kale G, Haliloglu M, Kiper N (2009) Successful unilateral partial lung lavage in a child with pulmonary alveolar proteinosis. J Clin Anesth 21:127–130

    Article  PubMed  Google Scholar 

  32. Emiralioglu N, Beken B, Ozcan HN, Yalcin E, Dogru D, Ozcelik U, Haliloglu M, Kiper N (2016) Diagnosis and treatment of pulmonary alveolar microlithiasis. Pediatr Int 58:805–807

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgements

We thank all the families and patients for their participation. We thank all the chILD-TR collaborators.

Funding

This project was funded by Hacettepe University Scientific Research Project Coordination Department (Project number: TOA-2021–19075).

Author information

Authors and Affiliations

  1. Division of Pulmonology, Department of Pediatrics, Faculty of Medicine, Hacettepe University, Ankara, Turkey

    Halime Nayır-Büyükşahin, Nagehan Emiralioğlu, Ebru Yalçın, Deniz Doğru, Uğur Özçelik & Nural Kiper

  2. Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, Ihsan Dogramaci Children’s Hospital, Ankara, Turkey

    Halime Nayır-Büyükşahin

  3. Division of Pulmonology, Department of Pediatrics, Cerrahpaşa Faculty of Medicine Faculty of Medicine, İstanbul University-Cerrahpaşa, Istanbul, Turkey

    Ayşe Ayzıt Kılınç, Azer Kılıç Başkan, Hüseyin Arslan & Haluk Çokuğraş

  4. Division of Pulmonology, Department of Pediatrics, Faculty of Medicine, Istanbul Medeniyet University, Istanbul, Turkey

    Saniye Girit, Zeynep Reyhan Onay, Sinem Can Oksay & Deniz Mavi Tortop

  5. Division of Pulmonology, Department of Pediatrics, Faculty of Medicine, Gazi University, Ankara, Turkey

    Tuğba Şişmanlar Eyüboğlu, Ayşe Tana Aslan & Handan Kekeç

  6. Division of Pulmonology, Department of Pediatrics, Faculty of Medicine, Ankara University, Ankara, Turkey

    Nazan Çobanoğlu, Fazılcan Zirek & Merve Nur Tekin

  7. Division of Pulmonology, Department of Pediatrics, Ankara City Hospital, Ankara, Turkey

    Güzin Cinel, Sanem Eryılmaz Polat & Salih Uytun

  8. Division of Pulmonology, Department of Pediatrics, Faculty of Medicine, Necmettin Erbakan University, Konya, Turkey

    Sevgi Pekcan, Aslı İmran Yılmaz & Gökçen Ünal

  9. Division of Pulmonology, Department of Pediatrics, Faculty of Medicine, Marmara University, Istanbul, Turkey

    Yasemin Gökdemir, Ela Erdem Eralp & Bülent Karadağ

  10. Division of Pediatric Radiology, Department of Radiology, Faculty of Medicine, Hacettepe University, Ankara, Turkey

    Berna Oğuz

  11. Department of Pathology, Faculty of Medicine, Hacettepe University, Ankara, Turkey

    Diclehan Orhan

  12. Division of Pulmonology, Department of Pediatrics, Faculty of Medicine, Ege University, İzmir, Turkey

    Figen Gülen & Bahar Girgin Dindar

  13. Division of Pediatric Pulmonology, Gaziantep Cengiz Gökçek Maternity and Children Hospital, Gaziantep, Turkey

    Melih Hangül

  14. Division of Pulmonology, Department of Pediatrics, Faculty of Medicine, Erciyes University, Kayseri, Turkey

    Mehmet Köse

  15. Division of Pediatric Pulmonology, Şanlıurfa Children Hospital, Şanıurfa, Turkey

    Beste Özsezen

  16. Division of Pulmonology, Department of Pediatrics, Faculty of Medicine, İstinye University, Istanbul, Turkey

    Erkan Çakır

  17. Department of Pediatric Pulmonology, Allergy and Immunology, Faculty of Medicine, Akdeniz University, Antalya, Turkey

    Ayşen Bingöl

  18. Department of Pediatric Pulmonology, Ankara Training and Research Hospital, University of Health Science, Ankara, Turkey

    Mina Hızal

  19. Division of Pulmonology, Department of Pediatrics, University of Health Sciences Dr. Behçet Uz Child Disease and Pediatric Surgery Training and Research Hospital, İzmir, Turkey

    Gökçen Kartal Öztürk

  20. Division of Pediatric Pulmonology, Faculty of Medicine, Koc University, Istanbul, Turkey

    Zeynep Seda Uyan

  21. Division of Pediatric Pulmonology, Dr Sami Ulus Maternity and Children Training and Research Hospital, Ankara, Turkey

    Tuğba Ramaslı Gürsoy

  22. Division of Pediatric Allergy and Immunology, Department of Pediatrics, Faculty of Medicine, Çukurova University, Adana, Turkey

    Mahir Serbes

Authors
  1. Halime Nayır-Büyükşahin
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Nagehan Emiralioğlu
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Ayşe Ayzıt Kılınç
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Saniye Girit
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Ebru Yalçın
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Tuğba Şişmanlar Eyüboğlu
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Nazan Çobanoğlu
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Güzin Cinel
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Sevgi Pekcan
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Yasemin Gökdemir
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Berna Oğuz
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Diclehan Orhan
    View author publications

    You can also search for this author in PubMed Google Scholar

  13. Deniz Doğru
    View author publications

    You can also search for this author in PubMed Google Scholar

  14. Uğur Özçelik
    View author publications

    You can also search for this author in PubMed Google Scholar

  15. Azer Kılıç Başkan
    View author publications

    You can also search for this author in PubMed Google Scholar

  16. Hüseyin Arslan
    View author publications

    You can also search for this author in PubMed Google Scholar

  17. Haluk Çokuğraş
    View author publications

    You can also search for this author in PubMed Google Scholar

  18. Zeynep Reyhan Onay
    View author publications

    You can also search for this author in PubMed Google Scholar

  19. Sinem Can Oksay
    View author publications

    You can also search for this author in PubMed Google Scholar

  20. Deniz Mavi Tortop
    View author publications

    You can also search for this author in PubMed Google Scholar

  21. Ayşe Tana Aslan
    View author publications

    You can also search for this author in PubMed Google Scholar

  22. Handan Kekeç
    View author publications

    You can also search for this author in PubMed Google Scholar

  23. Fazılcan Zirek
    View author publications

    You can also search for this author in PubMed Google Scholar

  24. Merve Nur Tekin
    View author publications

    You can also search for this author in PubMed Google Scholar

  25. Figen Gülen
    View author publications

    You can also search for this author in PubMed Google Scholar

  26. Bahar Girgin Dindar
    View author publications

    You can also search for this author in PubMed Google Scholar

  27. Sanem Eryılmaz Polat
    View author publications

    You can also search for this author in PubMed Google Scholar

  28. Salih Uytun
    View author publications

    You can also search for this author in PubMed Google Scholar

  29. Aslı İmran Yılmaz
    View author publications

    You can also search for this author in PubMed Google Scholar

  30. Gökçen Ünal
    View author publications

    You can also search for this author in PubMed Google Scholar

  31. Ela Erdem Eralp
    View author publications

    You can also search for this author in PubMed Google Scholar

  32. Bülent Karadağ
    View author publications

    You can also search for this author in PubMed Google Scholar

  33. Melih Hangül
    View author publications

    You can also search for this author in PubMed Google Scholar

  34. Mehmet Köse
    View author publications

    You can also search for this author in PubMed Google Scholar

  35. Beste Özsezen
    View author publications

    You can also search for this author in PubMed Google Scholar

  36. Erkan Çakır
    View author publications

    You can also search for this author in PubMed Google Scholar

  37. Ayşen Bingöl
    View author publications

    You can also search for this author in PubMed Google Scholar

  38. Mina Hızal
    View author publications

    You can also search for this author in PubMed Google Scholar

  39. Gökçen Kartal Öztürk
    View author publications

    You can also search for this author in PubMed Google Scholar

  40. Zeynep Seda Uyan
    View author publications

    You can also search for this author in PubMed Google Scholar

  41. Tuğba Ramaslı Gürsoy
    View author publications

    You can also search for this author in PubMed Google Scholar

  42. Mahir Serbes
    View author publications

    You can also search for this author in PubMed Google Scholar

  43. Nural Kiper
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

H.N.B., N.E., and N.K. wrote the main manuscript text. All authors contributed data and reviewed the manuscript.

Corresponding author

Correspondence to Halime Nayır-Büyükşahin.

Ethics declarations

Ethics approval

The establishment of the national registry and data input was approved by the local ethics committee (Hacettepe University Ethics Board, reference numbers: GO 20/604), in addition to the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards.

Competing interests

The authors declare no competing interests.

Additional information

Communicated by Peter de Winter

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Nayır-Büyükşahin, H., Emiralioğlu, N., Kılınç, A.A. et al. Childhood interstitial lung disease in Turkey: first data from the national registry. Eur J Pediatr 183, 295–304 (2024). https://doi.org/10.1007/s00431-023-05290-9

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00431-023-05290-9

Keywords

Search

Navigation