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of Pediatric Gastroenterology, Hartford Hospital, Farmington, Connecticut 06115.The pathogenesis of acute fatty liver of pregnancy is unknown, but similarities in the clinical presentation and the histological appearance of the liver with those found in children with metabolic liverdisorders in pregnancy defects in the intramitochondrial beta-oxidation pathway of the liver suggest that a liverdisorders in pregnancy disturbance in hepatic fatty acid oxidation may play a role. We report a woman with acute fatty liver of pregnancy who gave birth to a liverdisorders in pregnancy seemingly normal full-term infant who was seen at 4 mo of age with hypoglycemia, coma and profound hepatic steatosis. The infant had a defect in fatty acid oxidation, long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency, and the mother proved to be heterozygous for this metabolic condition.
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