ACUTE FATTY LIVER IN PREGNANCY

Background
Acute fatty liver of pregnancy (AFLP) is a serious complication unique to pregnancy first
described by Sheehan in 1940.[1] It is characterized by microvesicular steatosis in the liver. The
foremost cause of AFLP is thought to be due to a mitochondrial dysfunction in the oxidation of
fatty acids leading to an accumulation in hepatocytes. The infiltration of fatty acids causes acute
liver insufficiency, which leads to most of the symptoms that present in this condition. If not
diagnosed and treated promptly, AFLP can result in high maternal and neonatal morbidity and
mortality.

Pathophysiology
The exact pathophysiology of AFLP is unknown. AFLP is unique to pregnancy. There does not
appear to be a predilection for any geographical area or race. It appears to occur more commonly
in primiparous women than multiparous women.
Women who develop AFLP are more likely to have a heterozygous long-chain 3-hydroxyacylcoenzyme A dehydrogenase (LCHAD) deficiency. LCHAD is found on the mitochondrial
membrane and is involved in the beta oxidation of long-chain fatty acids. This gene mutation is
recessive; therefore, outside of pregnancy under normal physiological conditions, women have
normal fatty acid oxidation. However, if the fetus is homozygous for this mutation, it will be
unable to oxidize fatty acids. These acids are passed to the mother, who, because of diminished
enzyme function, cannot metabolize the additional fatty acids. This results in hepatic strain
leading to the development of AFLP, which can be relieved by delivery of the infant.[2, 3]

Epidemiology
Frequency
United States
AFLP affects 1 in 7000 to 1 in 16,000 deliveries. There is a predilection for nulliparous women
and women with multiple gestations.

Mortality/Morbidity
Due to advances in diagnostic strategies and supportive care, maternal mortality and perinatal
morbidity of AFLP has declined. In the 1980s, Kaplan reported a mortality rate for both mother
and fetus of about 85%.[4] Maternal mortality is now estimated to be 12.5-18%, with a neonatal
mortality rate of 7-66%.[5, 6]

While laboratory abnormalities may persist after delivery, in rare cases patients may progress to
hepatic failure with the need for liver transplantation.[7]
Morbidity of the infant includes increased risk of cardiomyopathy, neuropathy, myopathy,
nonketotic hypoglycemia, hepatic failure, and death associated with fatty acid oxidation defects
in newborns.

Race
No ethnic or regional variability is apparent.

Sex
This condition is unique to pregnancy and therefore only affects women.

Age
This condition can affect any woman of child-bearing age.