Acute Fatty Liver of Pregnancy (AFLP): What You Need to Know

Acute fatty liver of pregnancy (AFLP) is a rare condition in which too much fat accumulates in the liver. If detected early enough, the damage is minimal. However, left untreated or poorly treated the condition can cause liver failure and even death. Although AFLP is rare, it cannot be predicted or prevented. In the past, the condition was almost always fatal. However, thanks to medical advances, mortality from AFLP has been greatly reduced.

Who gets AFLP?

AFLP is a rare condition affecting one in every 7,000 to 11,000 pregnancies. It normally occurs during the last trimester of pregnancy but can occur any time during the second half of pregnancy (after twenty weeks gestation.) However, it can also occur during the period immediately following delivery. AFLP most often affects first-time pregnancies. Although the condition does not usually affect subsequent pregnancies, in some cases AFLP can recur.

What causes AFLP?

The exact cause of the condition is not known. In many cases, however, AFLP has been found to be associated with an abnormality of fatty-acid metabolism as a result of a deficiency in LCHAD. The deficiency of LCHAD results in the accumulation of fatty acid in the liver. When this occurs in the fetus, it brings on the disease in the mother. In such cases, the disease is known to be genetic, following autosomal recessive pattern. Because its cause is known, genetic testing can take place to possibly prevent the disease. However, this form of the condition only occurs in a fraction of AFLP cases. In the remainder of AFLP cases, the cause is unknown.

What are the symptoms of AFLP?

Symptoms of AFLP can vary greatly. Sometimes they can even go unnoticed until delivery. Symptoms can include but are not limited to: vomiting, jaundice, abdominal pain, malaise, headache, tiredness, confusion, nausea, and pruritis. Although they are not true "symptoms," AFLP has also been found to be associated with other conditions including preeclampsia and diabetes insipidus. Patients with these conditions should be aware of the symptoms of AFLP and contact their doctor immediately if they think they are experiencing any.

How is AFLP diagnosed?

In order to diagnose the condition, a doctor will first listen to the symptoms the patient is describing, if any. He/she will also look for certain signs of AFLP such as encephalopathy, polydipsia, and ascites. If he/she suspects AFLP, then certain tests may be performed to confirm diagnosis and rule out other conditions. One test that may be performed is a liver biopsy. Another is an ultrasound which is used to look for fat build up in the liver. Blood tests are also used to check for elevated liver enzymes and low blood sugar, both signs of AFLP.

How is AFLP treated?

In most cases, once AFLP has been diagnosed, hospitalization and prompt delivery immediately follow. If the mother is unstable, she is usually stabilized before the baby is born. Although the baby can be born vaginally, it is usually done via cesarean section because AFLP poses an increased risk of bleeding to the mother. Depending on the severity of the condition, the mother may need to be in intensive care anywhere from a few hours to a few weeks. In general, treatment is aimed at combating and preventing the many complications of AFLP following delivery. These include cerebral edema, renal failure, hypoglycemia, infections, and hemorrhage. Although the condition usually improves after delivery, there have been some cases where marked deterioration follows delivery. Subsequent treatment depends on the severity of the condition. In rare cases, a liver transplant is necessary.

What is the prognosis of those with AFLP?

The prognosis for mother and infant depends on the severity of the disease. The mortality rate for mothers with the condition has been dramatically reduced in the last decade. It now stands at eighteen percent. Most of the cases of mortality are the result of complications of AFLP such as hemorrhage but not AFLP itself. Likewise, the mortality rate for infants born to mothers with AFLP has been reduced and now stands at twenty-three percent. Most cases of infant mortality are believed to be the result of premature delivery. In the past, AFLP was almost always fatal. Now, thanks to medical advances, mortality from the condition has been greatly reduced.

Sources

MedicineNet

Wikipedia