Acute fatty liver of pregnancy in a patient with chronic active hepatitis and associated hepatocyte alpha 1-antitrypsin inclusions.

Abstract

Acute fatty liver of pregnancy is a rare, potentially fatal condition that shares many of the signs and symptoms of severe preeclampsia. Early reports of this condition noted alarmingly high fetal and maternal mortality rates. However, recent reports have described more favorable outcomes due to prompt recognition and aggressive treatment. We here describe a patient with acute fatty liver of pregnancy superimposed on chronic active hepatitis. A 27-year-old woman, para 0-1-2-1, presented at 36 weeks' gestation with a 2-week history of malaise, nausea, emesis, diarrhea, and an 18-lb weight loss. The serum concentrations of transaminase enzymes, bilirubin, and alkaline phosphatase were increased, and a mild coagulopathy was present. The patient underwent repeat cesarean delivery and intraoperative needle biopsy of the liver. Histologic examination of the biopsy demonstrated the characteristic changes of acute fatty liver of pregnancy and chronic active hepatitis with associated alpha 1-antitrypsin hepatocyte inclusions. The serum alpha 1-antitrypsin level was mildly elevated and the phenotype was normal (PiMM), excluding alpha 1-antitrypsin deficiency. Viral and autoimmune etiologies for chronic hepatitis were excluded by laboratory studies. The woman experienced rapid resolution of symptoms and laboratory abnormalities in the immediate postoperative period. Acute fatty liver of pregnancy may occur in patients with underlying chronic liver disease. Prompt delivery is necessary to reduce the risk of fetal and maternal mortality.