In more patients with more severe disease, pre-eclampsia may occur, which involves elevation of blood pressure and accumulation of fluid (termed edema). This may progress to involvement of additional systems, including acute renal failure, hepatic encephalopathy, and pancreatitis. There have also been reports of diabetes insipidus complicating this condition.
Many laboratory abnormalities are seen in acute fatty liver of pregnancy. Liver enzymes are elevated, with the AST and ALT enzymes ranging from minimal elevation to 1000 IU/L, but usually staying in the 300-500 range. Bilirubin is almost universally elevated. Alkaline phosphatase is often elevated in pregnancy due to production from the placenta, but may be additionally elevated. Other abnormalities may include an elevated white blood cell count, hypoglycemia, elevated coagulation parameters, including the international normalized ratio, and decreased fibrinogen. Frank disseminated intravascular coagulation, or DIC, may occur in as many as 70% of patients.
Abdominal ultrasound may show fat deposition in the liver, but, as the hallmark of this condition is microvesicular steatosis (see pathology below), this may not be seen on ultrasound. Rarely, the condition can be complicated by rupture or necrosis of the liver, which may be identified by ultrasound.
A liver biopsy can provide a definitive diagnosis, but is not always done, due to the increased chance of bleeding in acute fatty liver of pregnancy. Often testing will be done to exclude more common conditions that present in a similar fashion, including viral hepatitis, pre-eclampsia, HELLP syndrome, intrahepatic cholestasis of pregnancy, and autoimmune hepatitis.
Initial treatment involves supportive management with intravenous fluids, intravenous glucose and blood products, including fresh frozen plasma and cryoprecipitate to correct DIC. The fetus should be monitored with cardiotocography. After the mother is stabilized, arrangements are usually made for delivery. This may occur vaginally, but, in cases of severe bleeding or compromise of the mother's status, a caesarian section may be needed.
The complications of acute fatty liver of pregnancy may require treatment after delivery, especially if pancreatitis occurs. Liver transplantation is rarely required for treatment of the condition, but may be needed for mothers with severe DIC, those with rupture of the liver, or those with severe encephalopathy.
The understanding of the causes of acute fatty liver of pregnancy has been ameliorated by advances in mitochondrial biochemistry. Deficiency of LCHAD (3-hydroxyacyl-CoA dehydrogenase) leads to an accumulation of medium and long chain fatty acids. When this occurs in the fetus, the unmetabolized fatty acids will re-enter the maternal circulation through the placenta, and overwhelm the beta-oxidation enzymes of the mother. The gene responsible for LCHAD has been isolated, and the most common mutation found in acute fatty liver of pregnancy is the E474Q missense mutation. The disease is autosomal recessive in inheritance and mothers are often found to be heterozygous for the affected mutation.
It's Greek to Me: More Than One Hundred Different Types of Ceramic Vessels Were Used in Ancient Greece. the Shapes and Functions of the Pieces Varied. the Finest of These Vessels Were Always Decorated
Nov 01, 2005; Initially, forms were incised into the clay. In about 530 BC a method called the red figure technique was invented, whereby the...
UK Activities: How to Turn Base Talents into Gold with Just a Thwack ; Armed with a Hammer, and with Much Bashing of Metal, Hester Lacey Spent a Day Making Jewellery
Aug 15, 2004; I had always thought that jewellery- making was rather a ladylike form of craftwork. I'd imagined delicately selecting a stone...