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

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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.