title: Kidney and liver transplantation in children with fibrocystic liver-kidney disease: data from the US Scientific Registry of Transplant Recipients: 1990-2010.
abstract: 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.
authors: Division of Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
authors: Jessica W
authors: JW
authors: Wen
authors: Susan L
authors: SL
authors: Furth
authors: Rebecca L
authors: RL
authors: Ruebner
mesh_terms: D000293:Adolescent; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D052177:Kidney Diseases, Cystic / Q000151:congenital / Q000601:surgery; D007676:Kidney Failure, Chronic / Q000601:surgery*; D016030:Kidney Transplantation / Q000706:statistics & numerical data*; D017093:Liver Failure / Q000601:surgery*; D016031:Liver Transplantation / Q000706:statistics & numerical data*; D008297:Male; D007690:Polycystic Kidney Diseases / Q000601:surgery*; D012042:Registries; D012086:Reoperation; D012189:Retrospective Studies; D016896:Treatment Outcome; D014481:United States
keywords: Caroli's syndrome; autosomal recessive polycystic kidney; congenital hepatic fibrosis; kidney transplantation; liver fibrocystic disease; liver transplantation; pediatric