Pregnancy and fetal long-chain 3-hydroxyacyl coenzyme A dehydrogenase deficiency

Lancet. 1993 Feb 13;341(8842):407-8. doi: 10.1016/0140-6736(93)92993-4.


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.

MeSH terms

  • 3-Hydroxyacyl CoA Dehydrogenases / deficiency*
  • Female
  • Fetal Diseases*
  • HELLP Syndrome / complications
  • Humans
  • Infant, Newborn
  • Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase
  • Pregnancy
  • Pregnancy Complications*


  • 3-Hydroxyacyl CoA Dehydrogenases
  • Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase