TY - JOUR
T1 - Kidney and liver transplantation in children with fibrocystic liver-kidney disease
T2 - Data from the US Scientific Registry of Transplant Recipients: 1990-2010
AU - Wen, Jessica W.
AU - Furth, Susan L.
AU - Ruebner, Rebecca L.
N1 - Publisher Copyright:
© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
PY - 2014
Y1 - 2014
N2 - The natural history and survival of children with fibrocystic liver-kidney disease undergoing solid organ transplantation have infrequently been described. We report outcomes in a cohort of US children with fibrocystic liver-kidney disease receiving solid organ transplants over 20 yr. Retrospective cohort study of pediatric transplant recipients with diagnoses of fibrocystic liver-kidney disease from 1/1990 to 3/2010, using data from the SRTR. Subjects were categorized by the first transplanted organ: LT, KT, or SLK. Primary outcomes were death, re-transplant, transplant of the alternate organ, or initiation of dialysis. Seven hundred and sixteen subjects were transplanted in this period. Median age at first transplant was 9.7 yr. Of the LT, 14 (19%) required a second liver transplant at median of 0.2 yr, and five (7%) required kidney transplant or dialysis at a median of 9.0 yr. Of the KT, 188 (31%) required a second kidney transplant or dialysis at a median of 5.9 yr. Twenty-nine (5%) subsequently received liver transplant at a median of 6.0 yr. Among patients in this registry, far more children underwent kidney than liver transplants. The risk of subsequently needing transplantation of an alternate organ was low.
AB - The natural history and survival of children with fibrocystic liver-kidney disease undergoing solid organ transplantation have infrequently been described. We report outcomes in a cohort of US children with fibrocystic liver-kidney disease receiving solid organ transplants over 20 yr. Retrospective cohort study of pediatric transplant recipients with diagnoses of fibrocystic liver-kidney disease from 1/1990 to 3/2010, using data from the SRTR. Subjects were categorized by the first transplanted organ: LT, KT, or SLK. Primary outcomes were death, re-transplant, transplant of the alternate organ, or initiation of dialysis. Seven hundred and sixteen subjects were transplanted in this period. Median age at first transplant was 9.7 yr. Of the LT, 14 (19%) required a second liver transplant at median of 0.2 yr, and five (7%) required kidney transplant or dialysis at a median of 9.0 yr. Of the KT, 188 (31%) required a second kidney transplant or dialysis at a median of 5.9 yr. Twenty-nine (5%) subsequently received liver transplant at a median of 6.0 yr. Among patients in this registry, far more children underwent kidney than liver transplants. The risk of subsequently needing transplantation of an alternate organ was low.
KW - Autosomal recessive polycystic kidney
KW - Caroli's syndrome
KW - Congenital hepatic fibrosis
KW - Kidney transplantation
KW - Liver fibrocystic disease
KW - Liver transplantation
KW - Pediatric
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UR - http://www.scopus.com/inward/citedby.url?scp=84926185563&partnerID=8YFLogxK
U2 - 10.1111/petr.12330
DO - 10.1111/petr.12330
M3 - Article
C2 - 25074681
AN - SCOPUS:84926185563
SN - 1397-3142
VL - 18
SP - 726
EP - 732
JO - Pediatric transplantation
JF - Pediatric transplantation
IS - 7
ER -