Liver Disease in Pregnancy

To the Editor: We were surprised to find that the review of liver disease in pregnancy (Aug. 22 issue) 1 did not mention the recognized association between acute fatty liver of pregnancy and long-chain-3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency, 2 – 5 a disorder of mitochondrial fatty-acid oxidation. This association opens a new chapter in our understanding of the pathogenesis of diseases of pregnancy related to metabolic disorders. For example, the accumulation of phenylalanine in a mother with phenylketonuria can have a teratogenic effect on an unaffected fetus. A woman who is heterozygous for LCHAD deficiency and whose fetus has the disorder can have unexpected . . .