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Acute fatty liver of pregnancy and long-chain 3-hydroxyacyl–coenzyme A dehydrogenase deficiency

✍ Scribed by William R. Treem; Piero Rinaldo; Daniel E. Hale; Charles A. Stanley; David S. Millington; Jeffrey S. Hyams; Sandra Jackson; Douglass M. Turnbull

Publisher
John Wiley and Sons
Year
1994
Tongue
English
Weight
762 KB
Volume
19
Category
Article
ISSN
0270-9139

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✦ Synopsis

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 defects in the intramitochondrial fboxidation pathway of the liver suggest that a 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 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, longchain 3-hydroxyacyl-coenzye A dehydrogenase deficiency, and the mother proved to be heterozygous for this metabolic condition. We hypothesize that the interaction of an affected fetus with a female heterozygous for this defect in fatty acid oxidation in the late third trimester accounts for some cases of acute fatty liver of pregnancy. (HEPATOLOGY 1994; 19:339-345.)

Acute fatty liver of pregnancy (AFLPI was first described in 1940 by Sheehan as a clinical syndrome characterized by anorexia, nausea, vomiting, abdominal pain and jaundice begmning late in the third trimester of pregnancy (1). Untreated, it can progress to fulminant liver failure and result in the death of both the mother and the fetus. At biopsy or autopsy, the maternal liver shows striking microvesicular fatty change (2). Recent reviews have suggested that this disorder has a wider

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