Definition of long-chain-3-hydroxyacyl-CoA dehydrogenase def.

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Deficiency, long-chain-3-hydroxyacyl-CoA dehydrogenase: Acute fatty liver of pregnancy (AFLP) has been found to be associated in some cases with an abnormality of fatty-acid metabolism. This abnormality is a deficiency of the enzyme long-chain-3-hydroxyacyl-CoA dehydrogenase (LCHAD).

The mother (and father) have 50% of normal LCHAD activity and the fetus has no LCHAD activity.

The metabolic disease in the baby's liver apparently causes the fatty liver disease in the mother. In cases of AFLP due to LCHAD deficiency, there is a 25% (or greater) risk of AFLP in each pregnancy.