What are the other Names for this Condition? (Also known as/Synonyms)

• AFLP (Acute Fatty Liver of Pregnancy)
• Gestational Acute Fatty Liver

What is Acute Fatty Liver of Pregnancy? (Definition/Background Information)

• Acute Fatty Liver of Pregnancy (AFLP) 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
• The cause of Acute Fatty Liver of Pregnancy is not well understood, but genetics may play a role
• Symptoms generally begin in the third trimester and may include persistent nausea and vomiting, pain in the stomach or upper-right abdomen, malaise, jaundice and headache
• Without prompt treatment, Acute Fatty Liver of Pregnancy can lead to coma, organ failure or death of the mother and baby
• Treatment involves stabilizing the mother and delivery of the baby

(Source: Acute Fatty Liver of Pregnancy; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Who gets Acute Fatty Liver of Pregnancy? (Age and Sex Distribution)

• Acute Fatty Liver of Pregnancy is a rare disorder affecting pregnant women
• The disorder approximately affects 1 in 7,000 to 1 in 15,000 pregnant women, typically during the third trimester of pregnancy
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Acute Fatty Liver of Pregnancy? (Predisposing Factors)

• Deficiency in long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) may be an important risk factor, since this deficiency may lead to accumulation of medium-chain and long-chain fatty acids in the body
• Multiple gestations may also increase the risk for development of AFLP

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Acute Fatty Liver of Pregnancy? (Etiology)

The cause of Acute Fatty Liver of Pregnancy (AFLP) is not fully understood. It is reported that certain genetic factors may play a role in its development.

• 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 (LCHAD), an enzyme used to breakdown 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

(Source:Acute Fatty Liver of Pregnancy; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

What are the Signs and Symptoms of Acute Fatty Liver of Pregnancy?

Symptoms of Acute Fatty Liver of Pregnancy (AFLP) typically develop during the third trimester of pregnancy, and may include:

• Persistent nausea and vomiting
• Pain in the stomach or upper-right abdomen
• Malaise
• Jaundice
• Headache
• Tiredness
• Confusion

(Source: Acute Fatty Liver of Pregnancy; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

How is Acute Fatty Liver of Pregnancy Diagnosed?

• The diagnosis of Acute Fatty Liver of Pregnancy (AFLP) is usually made based on clinical findings, including 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

(Source: Acute Fatty Liver of Pregnancy; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Acute Fatty Liver of Pregnancy?

The complications of Acute Fatty Liver of Pregnancy may include:

• Collapse of the circulatory system
• Pancreatitis
• Bleeding in the gastro-intestinal tract
• Kidney failure
• Sepsis
• Fetal distress

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Acute Fatty Liver of Pregnancy Treated?

• Treatment of Acute Fatty Liver of Pregnancy (AFLP) involves stabilizing the mother and delivery of the baby

(Source: Acute Fatty Liver of Pregnancy; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

How can Acute Fatty Liver of Pregnancy be Prevented?

• Acute Fatty Liver of Pregnancy may not be preventable, if it stems from a genetic cause
• Active research is currently being performed to explore the possibilities for treatment and prevention of disorders such as Acute Fatty Liver of Pregnancy
• During pregnancy, regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Acute Fatty Liver of Pregnancy? (Outcomes/Resolutions)

• With an accurate diagnosis and prompt delivery of the baby, the outcome of Acute Fatty Liver of Pregnancy may be favorable
• Fetal distress and compromise may occur even when the mother is clinically stable, the reasons for which are unclear
• Maternal mortality rate due to AFLP is estimated to be about 12-18%, while the fetal demise rate is determined to be 23%
• In general, the neonatal mortality rate of babies born to mother with AFLP varies widely, and ranges between 7-66%

Additional and Relevant Useful Information for Acute Fatty Liver of Pregnancy:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/