Maternal acute fatty liver of pregnancy and the associated risk for long-chain 3-hydroxyacyl-coenzyme a dehydrogenase (LCHAD) deficiency in infants.

Acute fatty liver disease of pregnancy has been recognized as a clinical problem since the 1980s. In the past 8 years, the association of this disease with a genetic inborn error of metabolism in the infant has been recognized. Women who are heterozygous for this disorder are usually asymptomatic until the capacity of their livers to metabolize free fatty acids (FFA) is overwhelmed by a homozygous fetus. The inborn error of metabolism, long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency, may not be immediately recognizable in the infant. Symptoms in the infant are often triggered by an increased long-chain fatty acid load in the diet, or by illness that results in breakdown of endogenous fat. The following case study reviews the clinical pathophysiology of this perinatal health problem and highlights the priorities for the care of infants born to mothers with acute fatty liver disease of pregnancy.