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 language | English |
|---|---|
| Pages (from-to) | 649-655 |
| Journal | Pediatric Nephrology |
| Volume | 34 |
| Issue number | 4 |
| DOIs | |
| Publication status | Published - 2019 |
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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 journal › Article
}
TY - JOUR
T1 - Infants with congenital nephrotic syndrome have comparable outcomes to infants with other renal diseases
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
UR - http://www.scopus.com/inward/record.url?scp=85055962936&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85055962936&partnerID=8YFLogxK
U2 - 10.1007/s00467-018-4122-0
DO - 10.1007/s00467-018-4122-0
M3 - Article
VL - 34
SP - 649
EP - 655
JO - Pediatric Nephrology
JF - Pediatric Nephrology
SN - 0931-041X
IS - 4
ER -