Abstract
Summary: 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.
Speculation: The present observation suggests that damage to peroxisomes may be an element in acute liver dysfunction. Consequently, drugs that stimulate the production or regeneration of peroxisomes could possibly be of therapeutic interest in such conditions.
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Landrieu, P., François, B., Lyon, G. et al. Liver Peroxisome Damage during Acute Hepatic Failure in Partial Ornithine Transcarbamylase Deficiency. Pediatr Res 16, 977–981 (1982). https://doi.org/10.1203/00006450-198212000-00001
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DOI: https://doi.org/10.1203/00006450-198212000-00001
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