Acute fatty liver of pregnancy

Synonyms

2

Overview

Acute fatty liver of pregnancy is a rare and serious complication of pregnancy. It is characterized by a build-up of fat in the liver, which can lead to liver damage. It can be fatal without prompt diagnosis and treatment which involves delivering the baby as soon as possible. the cause of AFLP is not well understood, but genetics may play a role. The possible explanation is that a disordered metabolism of fatty acids by mitochondria in the mother, caused by long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency.  The condition was previously thought to be universally fatal, but aggressive treatment by stabilizing the mother with intravenous fluids and blood products in anticipation of early delivery has improved prognosis.

Symptoms

Symptoms of acute fatty liver of pregnancy typically develop during the third trimester of pregnancy. They may resemble those seen in HELLP syndrome. Pregnant women who experience any of these symptoms should immediately contact their healthcare provider:

  • Nausea
  • Vomiting
  • Confusion
  • Tiredness
  • Headache
  • Loss of appetite
  • Malaise
  • Abdominal pain or upper-right abdomen
  • Fever
  • Jaundice
  • Pre-eclampsia
  • Fluid build up
  • Increased blood pressure
  • Bleeding inside abdomen
  • Liver dysfunction
  • Increased liver enzymes
  • Increased bilirubin level
  • Increased alkaline phosphatase level
  • Increased white blood cell count
  • Low blood sugar
  • Reduced fibrinogen level
  • Fatty deposits in liver
  • Jaundice

Causes

The cause of acute fatty liver of pregnancy is not fully understood. Genetics may play a role. Recent studies indicate that AFLP may be the result of a mitochondrial dysfunction in the oxidation of fatty acids in the liver. More specifically, there may be a deficiency of long-chain 3-hydroxyacyl-CoA dehydrogenase, an enzyme used to break down long chain fatty acids. Babies with this deficiency can develop life-threatening liver, heart and neuromuscular problems unless they are started on a special low-fat formula. For this reason, it has been suggested that babies born to women with AFLP be tested for fatty acid oxidation disorders. Although the risk of AFLP in subsequent pregnancies is unknown, it can occur in future pregnancies, even if testing for long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency is negative.

Diagnosis

The diagnosis is usually made based on clinical findings, includng setting, presentation, and laboratory and imaging results. Laboratory tests that may be helpful include serum aminotransferases, serum bilirubin, coagulation studies, electrolytes, serum glucose, uric acid levels, creatinine and white blood count. Imaging tests are used primarily to exclude other diagnoses. Liver biopsy is diagnostic, but not always possible during pregnancy.

Treatment

Treatment involves stabilizing the mother and delivery of the baby.