Orthotopic liver transplantation for children with Alagille syndrome

Ronen Arnon, Rachel Annunziato, Tamir Miloh, Frederick Suchy, Arnond Sakworawich, Sogawa Hiroshi, Iyer Kishore, Nanda Kerkar

Research output: Contribution to journalArticlepeer-review

26 Scopus citations

Abstract

AGS is an inherited disorder involving the liver, heart, eyes, face, and skeleton. Aim: To determine the outcome of LT in children with AGS compared to those with BA. Methods: Children with AGS and BA who had a LT between 10/1987 and 5/2008 were identified from the UNOS database. Results: Of 11 467 children who received a liver transplant, 461 (4.0%) had AGS and 3056 (26.7%) had BA. One- and five-yr patient survival was significantly lower in patients with AGS in comparison with patients with BA (AGS; 82.9%, 78.4%, BA; 89.9%, 84%, respectively). Early death (<30 days from transplant) was significantly higher in AGS than in BA. One- and five-yr graft survival was significantly lower in AGS than in BA (AGS; 74.7%, 61.5%, BA; 81.6%, 70.0%, respectively). Death from graft failure, neurological, and cardiac complications was significantly higher in patients with AGS than in patients with BA. Serum creatinine at transplant, prior LT, and cold ischemic time >12 h were identified as risk factors for death. Conclusion: Children with AGS were older at the time of LT and their one- and five-yr patient and graft survival were significantly lower compared to BA. Risk factors for poor outcome in AGS after LT were identified.

Original languageEnglish (US)
Pages (from-to)622-628
Number of pages7
JournalPediatric Transplantation
Volume14
Issue number5
DOIs
StatePublished - Aug 2010
Externally publishedYes

Keywords

  • biliary
  • cholestasis
  • end-stage liver disease
  • liver transplantation
  • outcome
  • pediatric

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Transplantation

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