title: Pregnancy and fetal long-chain 3-hydroxyacyl coenzyme A dehydrogenase deficiency.
abstract: We report on eleven pregnancies in 5 mothers. 6 of the babies had long-chain 3-hydroxyacyl coenzyme A dehydrogenase (LCHAD) deficiency, and each of the pregnancies was complicated by features such as fatty liver and HELLP (haemolysis, elevated liver enzymes, low platelets) syndrome. By contrast, 3 of the mothers also gave birth to unaffected babies, and these pregnancies were largely uncomplicated. We conclude that there may be adverse effects on maternal liver function from a fetus with LCHAD deficiency. Heterozygosity in the mother cannot alone account for the adverse effects because of the segregation of these effects with fetal LCHAD status.
authors.affiliation: Oliver Latham Laboratory, New South Wales Health Department, Sydney, Australia.
authors.forename: B
authors.initials: B
authors.lastname: Wilcken
authors.forename: K C
authors.initials: KC
authors.lastname: Leung
authors.forename: J
authors.initials: J
authors.lastname: Hammond
authors.forename: R
authors.initials: R
authors.lastname: Kamath
authors.forename: J V
authors.initials: JV
authors.lastname: Leonard
mesh_terms: D015094:3-Hydroxyacyl CoA Dehydrogenases / Q000172:deficiency*; D005260:Female; D005315:Fetal Diseases; D017359:HELLP Syndrome / Q000150:complications; D006801:Humans; D007231:Infant, Newborn; D063989:Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase; D011247:Pregnancy; D011248:Pregnancy Complications
chemical_list: D015094:3-Hydroxyacyl CoA Dehydrogenases; D063989:Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase