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Lessons learned from the ESPN/ERA–EDTA Registry

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Abstract

End-stage renal disease (ESRD) in children is a medically challenging condition. Due to its rarity and special features, methodologically sound collaborative studies are required. In 2007, a new European registry of pediatric renal replacement therapy (RRT), the ESPN/ERA–EDTA Registry, was launched. In recent years, the Registry has provided comprehensive data on incidence, prevalence, patient characteristics, RRT modalities, and mortality in pediatric ESRD, along with relevant insights into cardiovascular risk, anemia, nutrition and growth, transplantation outcomes, and rare diseases. In this review, we describe the study design and structure underlying the ESPN/ERA–EDTA Registry, summarize the major research findings from more than 20 publications, and discuss current limitations and the future challenges to overcome.

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Author information

Authors and Affiliations

  1. Department of Pediatrics, Bordeaux University Hospital, Bordeaux, France

    Jérôme Harambat

  2. ESPN/ERA–EDTA Registry, Department of Medical Informatics, Academic Medical Center, Amsterdam, Netherlands

    Marjolein Bonthuis, Karlijn J. van Stralen & Kitty J. Jager

  3. Department of Pediatric Nephrology, Emma Children’s Hospital, Academic Medical Center, Amsterdam, Netherlands

    Jaap W. Groothoff

  4. Department of Pediatric Nephrology, University Children’s Hospital, Heidelberg, Germany

    Franz Schaefer

  5. Department of Pediatric Nephrology, Bristol Royal Hospital for Children, Bristol, UK

    E. Jane Tizard

  6. Dialysis Unit, Gaslini Children’s Hospital, Genoa, Italy

    Enrico Verrina

Authors
  1. Jérôme Harambat
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  2. Marjolein Bonthuis
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  3. Jaap W. Groothoff
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  4. Franz Schaefer
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  5. E. Jane Tizard
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  6. Enrico Verrina
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  7. Karlijn J. van Stralen
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  8. Kitty J. Jager
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Correspondence to Jérôme Harambat.

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Responses

1. a, c

2. b, d

3. d

4. a, e

5. e

Multiple choice questions (answers are provided in backmatter, following the reference list)

Multiple choice questions (answers are provided in backmatter, following the reference list)

  1. 1.

    A population-based registry (please select 2 answers):

    1. a.

      is an organized system that uses observational study methods to collect uniform data from a population defined by a particular disease

    2. b.

      must include both individuals with and without the disease

    3. c.

      allows an exhaustive registration of cases in order to provide reliable epidemiological data

    4. d.

      does not allow monitoring trends in incidence and prevalence of the disease over time

    5. e.

      is a superior methodology to randomized controlled trials in the hierarchy of evidence in therapy.

  2. 2.

    Registry data on patient characteristics, incidence and prevalence of RRT in Europe showed that (please select 2 answers):

    1. a.

      glomerulonephritis is the most common primary disease requiring RRT

    2. b.

      incidence of RRT has remained relatively stable around 6 pmarp in children <15 years

    3. c.

      incidence of pediatric RRT has constantly increased over the past 10 years

    4. d.

      considerable differences in incidence and prevalence exist between countries

    5. e.

      hemodialysis is the most common RRT modality among incident patients.

  3. 3.

    Patient survival on RRT (please select one answer):

    1. a.

      is the highest in the youngest age group (<5 years of age)

    2. b.

      is around 85 % at 4 years after the start of RRT

    3. c.

      is higher in children starting with dialysis than in preemptive kidney transplant recipients

    4. d.

      is >75 % at 5 years in those who started dialysis during the neonatal period

    5. e.

      is similar in Europe and in the USA.

  4. 4.

    Which of the following ESPN/ERA–EDA Registry findings regarding CKD complications are true? (please select two answers):

    1. a.

      high Hb levels are associated with low ferritin levels (25–50 ng/ml) among dialysis patients

    2. b.

      uncontrolled hypertension is more prevalent in PD than in HD patients

    3. c.

      hyperlipidemia is uncommon after successful kidney transplantation

    4. d.

      around a quarter of RRT children reach an adult height below the 3rd percentile

    5. e.

      more than one third of adolescents on RRT are overweight or obese.

  5. 5.

    In studies focusing on rare diseases, the ESPN/ERA–EDTA Registry found that (please select one answer):

    1. a.

      the onset of RRT for cystinosis has been delayed over time

    2. b.

      patient survival improved over time in children with cystinosis and in those with oxalosis

    3. c.

      kidney graft survival is poorer in children with oxalosis receiving a kidney transplantation alone as compared with liver–kidney transplantation

    4. d.

      the majority of patients with CAKUT who progress to ESRD will start RRT during adulthood

    5. e.

      all of the above.

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Harambat, J., Bonthuis, M., Groothoff, J.W. et al. Lessons learned from the ESPN/ERA–EDTA Registry. Pediatr Nephrol 31, 2055–2064 (2016). https://doi.org/10.1007/s00467-015-3238-8

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  • DOI: https://doi.org/10.1007/s00467-015-3238-8

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