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

On behalf of the ESPN Dialysis Working Group

Research output: Contribution to journalArticle

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.

Original languageEnglish
Pages (from-to)649-655
JournalPediatric Nephrology
Volume34
Issue number4
DOIs
Publication statusPublished - 2019

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Nephrotic Syndrome
Dialysis
Kidney
Peritonitis
Transplantation
Peritoneal Dialysis
Renal Dialysis
Nephrology
Chronic Renal Insufficiency
Chronic Kidney Failure
Registries
Catheters

Keywords

  • Complications
  • Congenital nephrotic syndrome
  • Infant dialysis
  • Outcome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Nephrology

Cite this

Infants with congenital nephrotic syndrome have comparable outcomes to infants with other renal diseases. / On behalf of the ESPN Dialysis Working Group.

In: Pediatric Nephrology, Vol. 34, No. 4, 2019, p. 649-655.

Research output: Contribution to journalArticle

On behalf of the ESPN Dialysis Working Group. / Infants with congenital nephrotic syndrome have comparable outcomes to infants with other renal diseases. In: Pediatric Nephrology. 2019 ; Vol. 34, No. 4. pp. 649-655.
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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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AU - On behalf of the ESPN Dialysis Working Group

AU - Dufek, Stephanie

AU - Ylinen, Elisa

AU - Trautmann, Agnes

AU - Alpay, Harika

AU - Ariceta, Gema

AU - Aufricht, Christoph

AU - Bacchetta, Justine

AU - Bakkaloglu, Sevcan

AU - Bayazit, Aysun

AU - Caliskan, Salim

AU - do Sameiro Faria, Maria

AU - Dursun, Ismail

AU - Ekim, Mesiha

AU - Jankauskiene, Augustina

AU - Klaus, Günter

AU - Paglialonga, Fabio

AU - Pasini, Andrea

AU - Printza, Nikoleta

AU - Conti, Valerie Said

AU - Schmitt, Claus Peter

AU - Stefanidis, Constantinos

AU - Verrina, Enrico

AU - Vidal, Enrico

AU - Webb, Hazel

AU - Zampetoglou, Argyroula

AU - Edefonti, Alberto

AU - Holtta, Tuula

AU - Shroff, Rukshana

PY - 2019

Y1 - 2019

N2 - 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.

AB - 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.

KW - Complications

KW - Congenital nephrotic syndrome

KW - Infant dialysis

KW - Outcome

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