Acute fatty liver of pregnancy

Acute fatty liver of pregnancy (AFLP), also known as acute fatty metamorphosis of pregnancy or obstetric acute yellow atrophy, is a rare but potentially life-threatening complication that occurs during the third trimester of pregnancy or in the early postpartum period. It is characterized by microvesicular fatty infiltration of hepatocytes (liver cells), leading to progressive liver failure. The condition primarily affects the liver but can rapidly progress to involve multiple organ systems, including the kidneys, pancreas, and coagulation system, potentially causing disseminated intravascular coagulation (DIC), renal failure, and encephalopathy. Key symptoms include nausea, vomiting, abdominal pain (particularly in the right upper quadrant or epigastric region), malaise, anorexia, and jaundice. Laboratory findings typically reveal elevated liver enzymes, hypoglycemia, elevated bilirubin, coagulopathy, leukocytosis, and sometimes hyperuricemia. In severe cases, patients may develop hepatic encephalopathy, ascites, and multi-organ failure. AFLP has been associated in some cases with an underlying deficiency of long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) in the fetus, a mitochondrial fatty acid oxidation disorder inherited in an autosomal recessive manner. Heterozygous mothers carrying a fetus homozygous or compound heterozygous for LCHAD mutations may be at increased risk. The cornerstone of treatment is prompt delivery of the baby, which typically leads to resolution of the maternal liver disease. Supportive care is critical and may include correction of hypoglycemia, management of coagulopathy with fresh frozen plasma or cryoprecipitate, and intensive care monitoring. In rare, severe cases where liver failure does not resolve after delivery, liver transplantation may be considered. With early recognition and appropriate management, maternal and fetal outcomes have improved significantly, though AFLP remains associated with maternal mortality rates of approximately 1–12% and perinatal mortality rates of up to 9–23%. Screening of the newborn for fatty acid oxidation defects, particularly LCHAD deficiency, is recommended following a diagnosis of AFLP.

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