Liver peroxisome damage during acute hepatic failure in partial ornithine transcarbamylase deficiency

Abstract

A boy with ornithine transcarbamylase (OTC) deficiency was relatively symptom free for 9 months and then developed an acute episode with liver failure and metabolic imbalance. Subsequently there was severe cerebral atrophy. Liver ornithine transcarbamylase activity was 3% of the normal mean. Of considerable interest was the finding of an accelerated breakdown of liver peroxisomes during the acute phase.