gynecology and obstetrics
Hepatic Disorders in Pregnancy
Fatty liver of pregnancy
This rare, poorly understood disorder occurs near term, sometimes with Preeclampsia and Eclampsia . Patients may have an inherited defect in mitochondrial fatty acid beta-oxidation (which provides energy for skeletal and cardiac muscle); risk of fatty liver of pregnancy is 20 times higher in women with a mutation affecting long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD), particularly the G1528C mutation on one or both alleles (autosomally inherited). Symptoms of fatty liver include acute nausea and vomiting, abdominal discomfort, and jaundice, followed in severe cases by rapidly progressive hepatocellular failure. Maternal and fetal mortality rates are high in severe cases. A seemingly identical disorder may develop at any stage of pregnancy if high doses of tetracyclines are given IV. Clinical and laboratory findings resemble those of fulminant viral hepatitis except that aminotransferase levels may be<500 units/L and hyperuricemia may be present. Diagnosis of fatty liver of pregnancy is based on Clinical criteria Liver tests Hepatitis serologic tests Liver biopsy Biopsy shows diffuse small droplets of fat in hepatocytes, usually with minimal apparent necrosis, but in some cases, findings are indistinguishable from viral hepatitis. Affected women and their infants should be tested for known genetic variants of LCHAD. Depending on gestational age, prompt delivery or termination of pregnancy is usually advised, although whether either alters maternal outcome is unclear. Survivors recover completely and have no recurrences. 