Alagille syndrome and liver transplantation

Binita M. Kamath, Kathleen B. Schwarz, Nedim Hadĩć

Research output: Contribution to journalReview article

Abstract

Alagille syndrome is a multisystem disorder in which progressive liver disease with persistent cholestasis and dramatic pruritus often warrant consideration for liver transplantation. The most important part of the transplant assessment is evaluation of the cardiac and renal involvement. Preoperatively, cardiac performance often must be tested with dynamic stress tests, mimicking hemodynamic changes during liver transplant. Many aspects of the syndrome including cholestasis, pruritus, and hypercholesterolemia improve posttransplant, but short stature is rarely significantly affected. One- and 5-year patient and graft survival after liver transplant is comparable to other elective indications, but effects of long-term immunosuppressants on evolution of other components of the syndrome, including vascular, bone, and renal disease, remain largely unknown.

Original languageEnglish (US)
Pages (from-to)11-15
Number of pages5
JournalJournal of pediatric gastroenterology and nutrition
Volume50
Issue number1
DOIs
StatePublished - Jan 1 2010

    Fingerprint

Keywords

  • Alagille syndrome
  • Cholestasis
  • Hypercholesterolemia
  • Pruritus

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Gastroenterology

Cite this