Acute Fatty Liver of Pregnancy

AFLP develops during the third trimester. It presents with vague symptoms, such as malaise, nausea, and vomiting. Severe cases present with jaundice, coagulopathy, hypoglycemia, and hyperammonemia. When undiagnosed, AFLP may progressively worsen to coma and death. Biochemical changes include mildly to moderately elevated AT levels, prolonged prothrombin time, and decreased fibrinogen level. Liver histology reveals microvesicular fatty infiltration, predominantly in the central region. Most cases of AFLP are managed with supportive measures, including transfer to an intensive care unit, mechanical ventilation, and correction of abnormal coagulation times. Severe cases may require prompt termination of pregnancy. Some women have been reported to experience recurrent AFLP in subsequent pregnancies (33).

The pathogenesis of AFLP remains unclear. Recent reports revealed that some women with AFLP are heterozygous for a defect in one of the enzymes (long-chain 3-hydroxyl-acyl CoA dehydrogenase [LCHAD]), involved in p oxidation of fatty acids in the mitochondria. These women remain asymptomatic until they become pregnant with a LCHAD homozygous fetus, and develop AFLP. Infants with LCHAD deficiency may develop a Reye's-like syndrome and possible death caused by nonke-totic hypoglycemia. A DNA test is available to identify the gene defect involved in LCHAD deficiency. This test allows for identification of heterozygous mothers who should receive counseling regarding subsequent pregnancies (34).

0 0

Post a comment