Acute fatty liver of pregnancy
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Acute fatty liver of pregnancy (AFLP) is a rare and potentially fatal complication that typically occurs in the last trimester or early postpartum period usually from unknown pathogenesis but has been linked to an abnormalits in fetal fatty acid metabolism. This abnormality is a deficiency in the LCHAD (long-chain 3-hydroxyacyl-coenzyme A dehydrogenase) enzyme.
Clinical manifestation usually manifests in the third trimester (35 to 36 weeks of gestation) but some cases occur with a rage of 28 to 40 weeks. Patients usually present with nonspecific symptoms such as anorexia, nausea, vomiting, malaise, fatigue, headache and abdominal pain. On physical examination, patients are febrile and jaundiced. Tenderness in the right upper abdomen usually present. In a severe form of disease, patient may present with multiple organ failure (renal, cardiac failure, GI bleeding) and some women also present with the symptoms of pre-eclampsia, eclampsia or HELLP syndrome.
On laboratory finding, patients will often have an elevated white blood count, but a normal hemoglobulin level. Thrombocytopenia may be present. Liver function tests shows elevated prothrombin and partial thromboplastic times, low fibrogen levels and elevated serum AST, ALT, ALP.
The diagnosis of acute fatty liver of pregnancy is challenging task for clinician because of the nonspecific clinical presentation which may mimic conditions such as acute viral hepatic, pre-eclampsia, HEELP syndrome. Ultrasound, CT, MRI may be used to diagnose this disease. Liver biopsy is the gold standard for diagnosed AFLP.
Early diagnosis, prompt delivery and intensive supportive care are the main stay in the management of AFLP. First patients are stabilized with intravenous fluid or blood products. After the mother is stabilized, delivery of the fetus is the next step. Liver transplantation may be the option for severe liver failure patients.
The mortality from AFLP is approximately 18% and deaths are usually secondary to sepsis, renal failure, circulatory collapse, pancreatitis or gastrointestinal bleeding.
Filed Under: Gastroenterology • Medicine
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