Acute fatty liver of pregnancy

HELLP syndrome is a thrombotic microangiopathy of pregnancy, a severe-feature variant of preeclampsia defined by hemolysis, transaminases at least twice the upper limit of normal, and a platelet count under 100,000/uL. Delivery is the only curative treatment; management pairs blood pressure control and magnesium sulfate seizure prophylaxis with prompt, gestational-age-guided delivery.

Intrahepatic cholestasis of pregnancy causes intractable pruritus of the palms and soles in the second to third trimester with elevated serum bile acids and carries a risk of sudden stillbirth late in gestation. First-line treatment is ursodeoxycholic acid to reduce pruritus and improve fetal outcome, with delivery planned for 36 to 38 weeks in proven disease.

New-onset hypertension after 20 weeks of gestation with proteinuria or end-organ dysfunction, driven by abnormal placental spiral artery remodeling and widespread endothelial dysfunction. Eclampsia is preeclampsia complicated by seizures; magnesium sulfate treats and prevents seizures, and prompt delivery is the only definitive cure for both.

Acute liver failure is the abrupt onset of hepatic synthetic dysfunction, defined by coagulopathy with an INR of 1.5 or higher plus any grade of hepatic encephalopathy, occurring within 26 weeks of illness onset in a patient without preexisting liver disease. Acetaminophen overdose is the leading cause in the United States, and management centers on N-acetylcysteine when acetaminophen is implicated, intensive supportive care, and early referral to a liver transplant center.

Disseminated intravascular coagulation is a secondary, systemic activation of coagulation and fibrinolysis, most often from sepsis, that simultaneously consumes platelets and clotting factors, causing microvascular thrombosis and bleeding. Diagnosis rests on a laboratory constellation, thrombocytopenia, prolonged PT and aPTT, low fibrinogen, and elevated D-dimer, in a predisposing clinical setting, and management centers on treating the underlying trigger while replacing blood components only for active or high-risk bleeding.

Hepatitis E virus is a fecal-oral or foodborne RNA virus with four genotypes that usually causes self-limited acute hepatitis resembling hepatitis A, but carries a high mortality risk in pregnant patients and can become chronic in immunocompromised hosts, especially solid-organ transplant recipients. Diagnosis rests on HEV IgM or RNA, treatment is supportive for acute disease and ribavirin for chronic infection, and prevention is safe water and thoroughly cooked pork or venison.

Wilson disease is an autosomal recessive disorder of the ATP7B copper-transporting ATPase causing toxic copper accumulation in the liver, brain, and cornea, presenting with hepatic and/or neuropsychiatric disease usually before age 40; diagnosis rests on ceruloplasmin, urinary copper, and Kayser-Fleischer rings, and lifelong treatment is chelation with penicillamine or trientine, or zinc, unless liver transplant is required.

Budd-Chiari syndrome is obstruction of hepatic venous outflow, usually hepatic vein thrombosis, causing hepatomegaly, ascites, and right-upper-quadrant pain. Diagnosis is by Doppler ultrasound; first-line treatment is anticoagulation, escalating to angioplasty, stenting, or TIPS for progressive disease.

Autoimmune hepatitis is a chronic inflammatory hepatitis, four times more common in women, marked by elevated aminotransferases, autoantibodies, and hypergammaglobulinemia, confirmed by liver biopsy after excluding other causes. Treatment is immunosuppression with prednisone, usually combined with azathioprine, continued for at least 2 to 3 years before withdrawal is considered.