A Fetal Fatty-Acid Oxidation Disorder as a Cause of Liver Disease in Pregnant Women
Open Access
- 3 June 1999
- journal article
- research article
- Published by Massachusetts Medical Society in New England Journal of Medicine
- Vol. 340 (22) , 1723-1731
- https://doi.org/10.1056/nejm199906033402204
Abstract
Acute fatty liver of pregnancy and the HELLP syndrome (hemolysis, elevated liver-enzyme levels, and a low platelet count) are serious hepatic disorders that may occur during pregnancy in women whose fetuses are later found to have a deficiency of long-chain 3-hydroxyacyl-coenzyme A (CoA) dehydrogenase. This enzyme resides in the mitochondrial trifunctional protein, which also contains the active site of long-chain 2,3-enoyl-CoA hydratase and long-chain 3-ketoacyl-CoA thiolase. We undertook this study to determine the relation between mutations in the trifunctional protein in infants with defects in fatty-acid oxidation and acute liver disease during pregnancy in their mothers.Keywords
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