ACUTE FATTY LIVER OF PREGNANCY AND LONG-CHAIN 3-HYDROXYACYL COENZYME-A DEHYDROGENASE-DEFICIENCY

The pathogenesis of acute fatty liver of pregnancy is unknown, but sim ilarities in the clinical presentation and the histological appearance of the liver with those found in children with metabolic defects in t he intramitochondrial beta-oxidation pathway of the liver suggest that a disturbance in hepatic fatty acid oxidation may play a role. We rep ort a woman with acute fatty liver of pregnancy who gave birth to a se emingly normal full-term infant who was seen at 4 mo of age with hypog lycemia, coma and profound hepatic steatosis. The infant had a defect in fatty acid oxidation, long-chain 3-hydroxyacyl-coenzyme A dehydroge nase deficiency, and the mother proved to be heterozygous for this met abolic condition. We hypothesize that the interaction of an affected f etus with a female heterozygous for this defect in fatty acid oxidatio n in the late third trimester accounts for some cases of acute fatty l iver of pregnancy.