Liver peroxisome damage during acute hepatic failure in partial ornithine transcarbamylase deficiency.
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.
D000208:Acute Disease; D000592:Amino Acid Metabolism, Inborn Errors / Q000201:enzymology* / Q000235:genetics / Q000473:pathology; D006501:Hepatic Encephalopathy / Q000201:enzymology* / Q000473:pathology; D006801:Humans; D007223:Infant; D008099:Liver / Q000201:enzymology / Q000473:pathology; D008111:Liver Function Tests; D008297:Male; D008830:Microbodies / Q000201:enzymology* / Q000648:ultrastructure; D009940:Organoids / Q000201:enzymology*; D020163:Ornithine Carbamoyltransferase Deficiency Disease