Mitochondrial fatty acid oxidation and acute fatty liver of pregnancy.

Accumulation of micro- and macrovesicular fat in hepatocytes is the hallmark of several liver diseases of pregnancy, including acute fatty liver of pregnancy (AFLP) and severe pre-eclampsia. While the exact origin of AFLP is not known, this pathologic finding and its similarity to the hepatopathology found in infants and children with inborn errors of mitochondrial fatty acid-oxidation suggests a common pathogenesis. Over the last few years, the incidence of AFLP and other severe complications of pregnancy have been found to be increased in women who carry a fetus with a defect in fatty acid oxidation, and who are themselves carriers of a genetic mutation that partially compromises their own intramitochondrial fatty acid oxidation pathway. This article reviews evidence that links AFLP with both genetic and acquired factors that compromise intramitochondrial fatty acid oxidation. Based on this review, recommendations are offered for obstetric, perinatal, and neonatal management of affected mothers and infants.