Kidney and liver transplantation in children with fibrocystic liver-kidney disease: Data from the US Scientific Registry of Transplant Recipients: 1990-2010

Jessica W. Wen, Susan L. Furth, Rebecca L. Ruebner

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
Pages (from-to)726-732
Number of pages7
JournalPediatric transplantation
Volume18
Issue number7
DOIs
StatePublished - Jan 1 2014

Keywords

  • Autosomal recessive polycystic kidney
  • Caroli's syndrome
  • Congenital hepatic fibrosis
  • Kidney transplantation
  • Liver fibrocystic disease
  • Liver transplantation
  • Pediatric

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Transplantation

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