Document Detail

Liver disease in pregnancy and fetal fatty acid oxidation defects.
MedLine Citation:
PMID:  11001809     Owner:  NLM     Status:  MEDLINE    
Acute fatty liver of pregnancy (AFLP) and the syndrome of hemolysis, elevated liver enzymes, and low platelets (the HELLP syndrome) are serious disorders of the third trimester with high maternal and perinatal morbidity and mortality. Over the past decade, several clinical observations have demonstrated an association between these maternal syndromes and a recessively inherited fatty acid oxidation disorder, long chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency. Many women who carried LCHAD-deficient fetuses developed maternal liver disease. Over the past few years, we and others have made significant progress in understanding the molecular basis for this fetal-maternal interaction. Here, we review the studies in literature that led to the establishment of this causative association with particular emphasis on the molecular analysis that delineated the molecular basis of this association. The likely mechanisms for the genotype-phenotype correlations in pediatric LCHAD deficiency and the fetal-maternal interaction are discussed. Finally, the potential implications of our current knowledge for families with pediatric LCHAD deficiency and for women who develop AFLP and HELLP syndrome are discussed.
J A Ibdah; Z Yang; M J Bennett
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.; Review    
Journal Detail:
Title:  Molecular genetics and metabolism     Volume:  71     ISSN:  1096-7192     ISO Abbreviation:  Mol. Genet. Metab.     Publication Date:    2000 Sep-Oct
Date Detail:
Created Date:  2000-11-13     Completed Date:  2000-11-13     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  9805456     Medline TA:  Mol Genet Metab     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  182-9     Citation Subset:  IM    
Copyright Information:
Copyright 2000 Academic Press.
Department of Internal Medicine, Wake Forest University School of Medicine, Winston-Salem, North Carolina 27157, USA.
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MeSH Terms
3-Hydroxyacyl CoA Dehydrogenases / deficiency,  genetics
Fatty Acids / metabolism*
Fatty Liver / complications*,  metabolism*
Fetus / metabolism
HELLP Syndrome / complications*,  metabolism*
Maternal-Fetal Exchange
Mitochondria, Liver / metabolism
Pregnancy Complications / metabolism*
Grant Support
Reg. No./Substance:
0/Fatty Acids; EC 3-hydroxyacyl CoA dehydrogenase; EC CoA Dehydrogenases

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