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29.10.2018 | Original Article | Ausgabe 4/2019

Pediatric Nephrology 4/2019

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

Zeitschrift:
Pediatric Nephrology > Ausgabe 4/2019
Autoren:
Stephanie Dufek, Elisa Ylinen, Agnes Trautmann, Harika Alpay, Gema Ariceta, Christoph Aufricht, Justine Bacchetta, Sevcan Bakkaloglu, Aysun Bayazit, Salim Caliskan, Maria do Sameiro Faria, Ismail Dursun, Mesiha Ekim, Augustina Jankauskiene, Günter Klaus, Fabio Paglialonga, Andrea Pasini, Nikoleta Printza, Valerie Said Conti, Claus Peter Schmitt, Constantinos Stefanidis, Enrico Verrina, Enrico Vidal, Hazel Webb, Argyroula Zampetoglou, Alberto Edefonti, Tuula Holtta, Rukshana Shroff, On behalf of the ESPN Dialysis Working Group
Wichtige Hinweise

Electronic supplementary material

The online version of this article (https://​doi.​org/​10.​1007/​s00467-018-4122-0) contains supplementary material, which is available to authorized users.

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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