Death after Transplantation of a Liver from a Donor with Unrecognized Ornithine Transcarbamylase Deficiency

Abstract

Ornithine transcarbamylase deficiency is the most common inherited disorder of the urea cycle.^1,2 It is an X-linked disease that can range in severity from hyperammonemic coma in neonates to the absence of symptoms in adults.^1,2 Liver transplantation is an effective treatment for patients with this disease.³ However, this disease has another implication for liver transplantation, as demonstrated by the following case.