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Acute fatty liver of pregnancy and long‐chain 3‐hydroxyacyl–coenzyme A dehydrogenase deficiency
Author(s) -
Treem William R.,
Rinaldo Piero,
Hale Daniel E.,
Stanley Charles A.,
Millington David S.,
Hyams Jeffrey S.,
Jackson Sandra,
Turnbull Douglass M.
Publication year - 1994
Publication title -
hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.488
H-Index - 361
eISSN - 1527-3350
pISSN - 0270-9139
DOI - 10.1002/hep.1840190211
Subject(s) - acute fatty liver of pregnancy , pregnancy , fatty liver , medicine , endocrinology , fetus , biology , disease , genetics
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 intra‐mitochondrial β‐oxidation 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, long‐chain 3‐hydroxyacyl–coenzyme 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).