Infants with congenital nephrotic syndrome have comparable outcomes to infants with other renal diseases.
Age Factors
Child, Preschool
Disease Progression
Europe
Female
Humans
Infant
Infant, Newborn
Kidney Failure, Chronic
/ diagnosis
Kidney Transplantation
/ adverse effects
Male
Nephrotic Syndrome
/ congenital
Peritoneal Dialysis
Renal Dialysis
/ adverse effects
Renal Insufficiency, Chronic
/ diagnosis
Retrospective Studies
Risk Factors
Time Factors
Treatment Outcome
Complications
Congenital nephrotic syndrome
Infant dialysis
Outcome
Journal
Pediatric nephrology (Berlin, Germany)
ISSN: 1432-198X
Titre abrégé: Pediatr Nephrol
Pays: Germany
ID NLM: 8708728
Informations de publication
Date de publication:
04 2019
04 2019
Historique:
received:
03
09
2018
accepted:
18
10
2018
revised:
12
10
2018
pubmed:
31
10
2018
medline:
28
4
2020
entrez:
31
10
2018
Statut:
ppublish
Résumé
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. 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. 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. 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.
Sections du résumé
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.
Identifiants
pubmed: 30374605
doi: 10.1007/s00467-018-4122-0
pii: 10.1007/s00467-018-4122-0
doi:
Types de publication
Comparative Study
Journal Article
Multicenter Study
Langues
eng
Sous-ensembles de citation
IM
Pagination
649-655Références
Nephrol Dial Transplant. 2019 Aug 1;34(8):1369-1377
pubmed: 30215773
Nephrol Dial Transplant. 2018 Aug 1;33(8):1459-1465
pubmed: 29617835
Eur J Pediatr. 2018 Jan;177(1):117-124
pubmed: 29143935
Am J Kidney Dis. 2017 May;69(5):617-625
pubmed: 27955924
Pediatr Nephrol. 2014 Jun;29(6):989-97
pubmed: 23812352
J Pediatr. 2009 Jul;155(1):111-7
pubmed: 19324367
Pediatr Nephrol. 2002 Aug;17(8):656-63
pubmed: 12185477
Pediatr Nephrol. 2004 Jan;19(1):82-90
pubmed: 14648343
Pediatr Nephrol. 2017 Dec;32(12):2319-2330
pubmed: 28762101
Pediatr Transplant. 1998 Nov;2(4):305-8
pubmed: 10084734
J Pediatr. 2000 Jan;136(1):24-9
pubmed: 10636969
Pediatr Nephrol. 2000 Oct;14(12):1077-82
pubmed: 11045390
Pediatr Nephrol. 1995 Feb;9(1):87-93
pubmed: 7742232
Pediatr Nephrol. 2016 Dec;31(12):2317-2325
pubmed: 27761660
Pediatr Nephrol. 2009 Nov;24(11):2121-8
pubmed: 17968594
Nephrol Dial Transplant. 2019 Mar 1;34(3):458-467
pubmed: 29474669
Nephrol Dial Transplant. 2012 Jan;27(1):388-95
pubmed: 21669887
Pediatr Nephrol. 2003 May;18(5):426-30
pubmed: 12687455
Pediatr Nephrol. 2016 May;31(5):833-41
pubmed: 26692024
Pediatr Nephrol. 2006 Feb;21(2):257-64
pubmed: 16270221
J Clin Invest. 2017 Mar 1;127(3):912-928
pubmed: 28165339
Perit Dial Int. 2016 9-10;36(5):481-508
pubmed: 27282851
Kidney Int Suppl. 1996 Jan;53:S51-6
pubmed: 8770991