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Infants with congenital nephrotic syndrome have comparable outcomes to infants with other renal diseases

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Abstract

Background

Children with congenital nephrotic syndrome (CNS) commonly develop end stage renal failure in infancy and require dialysis, but little is known about the complications and outcomes of dialysis in these children.

Methods

We conducted a retrospective case note review across members of the European Society for Pediatric Nephrology Dialysis Working Group to evaluate dialysis management, complications of dialysis, and outcomes in children with CNS.

Results

Eighty children (50% male) with CNS were identified form 17 centers over a 6-year period. Chronic dialysis was started in 44 (55%) children at a median age of 8 (interquartile range 4–14) months. Of these, 17 (39%) were on dialysis by the age of 6 months, 30 (68%) by 1 year, and 40 (91%) by 2 years. Peritoneal dialysis (PD) was the modality of choice in 93%, but 34% switched to hemodialysis (HD), largely due to catheter malfunction (n = 5) or peritonitis (n = 4). The peritonitis rate was 0.77 per patient-year. Weight and height SDS remained static after 6 months on dialysis. In the overall cohort, at final follow-up, 29 children were transplanted, 18 were still on dialysis (15 PD, 3 HD), 19 were in pre-dialysis chronic kidney disease (CKD), and there were 14 deaths (8 on dialysis). Median time on chronic dialysis until transplantation was 9 (6–18) months, and the median age at transplantation was 22 (14–28) months.

Conclusions

Infants with CNS on dialysis have a comparable mortality, peritonitis rate, growth, and time to transplantation as infants with other primary renal diseases reported in international registry data.

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Author information

Authors and Affiliations

  1. Great Ormond Street Hospital for Children NHS Foundation Trust, London, WC1N 3JH, UK

    Stephanie Dufek, Hazel Webb & Rukshana Shroff

  2. University of Helsinki and Helsinki University Hospital, Helsinki, Finland

    Elisa Ylinen & Tuula Holtta

  3. Center for Pediatric and Adolescent Medicine, Heidelberg, Germany

    Agnes Trautmann & Claus Peter Schmitt

  4. School of Medicine, Marmara University, Istanbul, Turkey

    Harika Alpay

  5. Hospital Universitari Vall d’Hebron. Universitat Autonoma de Barcelona, Barcelona, Spain

    Gema Ariceta

  6. Medical University of Vienna, Vienna, Austria

    Christoph Aufricht

  7. Hôpital Femme Mère Enfant, Lyon, France

    Justine Bacchetta

  8. Gazi University Hospital, Ankara, Turkey

    Sevcan Bakkaloglu

  9. Cukurova University, Adana, Turkey

    Aysun Bayazit

  10. Cerrahpasa Medical Faculty, Istanbul, Turkey

    Salim Caliskan

  11. Centro Materno Infantil do Norte, Porto, Portugal

    Maria do Sameiro Faria

  12. Erciyes University, Kayseri, Turkey

    Ismail Dursun

  13. Ankara University Hospital, Ankara, Turkey

    Mesiha Ekim

  14. Vilnius University, Vilnius, Lithuania

    Augustina Jankauskiene

  15. KfH Pediatric Kidney Center, Marburg, Germany

    Günter Klaus

  16. Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy

    Fabio Paglialonga & Alberto Edefonti

  17. Azienda Ospedaliero-Universitaria Sant’Orsola-Malpighi, Bologna, Italy

    Andrea Pasini

  18. 1st Pediatric Department, Aristotle University, Thessaloniki, Greece

    Nikoleta Printza

  19. Mater Dei Hospital Malta, Msida, Malta

    Valerie Said Conti

  20. “A & P Kyriakou”, Children’s Hospital, Athens, Greece

    Constantinos Stefanidis & Argyroula Zampetoglou

  21. IRCCS Giannina Gaslini, Genoa, Italy

    Enrico Verrina

  22. University-Hospital of Padova, Padova, Italy

    Enrico Vidal

Authors
  1. Stephanie Dufek
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  2. Elisa Ylinen
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  3. Agnes Trautmann
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  8. Sevcan Bakkaloglu
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  14. Augustina Jankauskiene
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  15. Günter Klaus
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  16. Fabio Paglialonga
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  17. Andrea Pasini
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  18. Nikoleta Printza
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  19. Valerie Said Conti
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  20. Claus Peter Schmitt
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  21. Constantinos Stefanidis
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  23. Enrico Vidal
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  26. Alberto Edefonti
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  27. Tuula Holtta
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  28. Rukshana Shroff
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On behalf of the ESPN Dialysis Working Group

Corresponding author

Correspondence to Rukshana Shroff.

Ethics declarations

Ethical approval for retrospective case-note review was obtained at each center as per local requirements.

Conflict of interest

The authors declare that they have no conflict of interest.

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Dufek, S., Ylinen, E., Trautmann, A. et al. Infants with congenital nephrotic syndrome have comparable outcomes to infants with other renal diseases. Pediatr Nephrol 34, 649–655 (2019). https://doi.org/10.1007/s00467-018-4122-0

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  • DOI: https://doi.org/10.1007/s00467-018-4122-0

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