Jaundice and disseminated intravascular coagulopathy in pregnancy.

Jaundice in pregnancy can be caused by viral hepatitis, intrahepatic cholestasis of pregnancy, choledocholithiases, HELLP syndrome (hemolysis, elevated liver enzymes, and a low platelet count), severe preeclampsia, and acute fatty liver of pregnancy. I Acute fatty liver of pregnancy occurs in approximately 1 in 13,000 pregnancies. More than 90% of patients with acute fatty liver of pregnancy have jaundice and disseminated intravascular coagulopathy (DIC).3 Before 1985 the associated maternal mortality was reported as 85%.4 Patients die with DIC, hemorrhage, severe hypoglycemia, and fulminant liver failure. Acute fatty liver of pregnancy was first recognized as a distinct clinical syndrome in 1940.5 The high maternal mortality initially associated with this condition was multifactorial, and in many cases the diagnosis was made at postmortem. Pregnancyrelated consumptive coagulopathy was not a recognized clinical entity until the 1950s. Furthermore, rapid serologic testing for infectious hepatitis was not readily available until the 1980s. The historic management of hepatitis in pregnancy was conservative, and patients with undiagnosed acute fatty liver of pregnancy frequently progressed to fulminant liver failure and death. Today early diagnosis of acute fatty liver of pregnancy and prompt delivery of the fetus is more commonplace. Rapid serologic testing for infectious hepatitis and DIC gives the modem clinician an edge in making the diagnosis. The following case has the classic clinical findings of acute fatty liver of pregnancy. The subsequent discussion provides an overview of this condition.