Presentation of an acquired urea cycle disorder post liver transplantation

Marwan Ghabril, Justin Nguyen, David Kramer, Trina Genco, Martin Mai, Barry G. Rosser

Research output: Contribution to journalArticle

5 Scopus citations

Abstract

The liver's role as the largest organ of metabolism and the unique and often critical function of liver-specific enzyme pathways imply a greater risk to the recipient of acquiring a donor metabolic disease with liver transplants versus other solid organ transplants. With clinical consequences rarely reported, the frequency of solid organ transplant transfer of metabolic disease is not known. Ornithine transcarbamylase deficiency (OTCD), although rare, is the most common of the urea cycle disorders (UCDs). Because of phenotypic heterogeneity, OTCD may go undiagnosed into adulthood. With over 5000 liver transplant procedures annually in the United States, the likelihood of unknowingly transmitting OTCD through liver transplantation is very low. We describe the clinical course of a liver transplant recipient presenting with acute hyperammonemia and encephalopathy after receiving a liver graft form a donor with unrecognized OTCD.

Original languageEnglish (US)
Pages (from-to)1714-1716
Number of pages3
JournalLiver Transplantation
Volume13
Issue number12
DOIs
StatePublished - Dec 1 2007

ASJC Scopus subject areas

  • Surgery
  • Hepatology
  • Transplantation

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