Fulminant acute fatty liver of pregnancy presenting with multi-organ failure: A case series.

Abstract

Liver disease in pregnancy can be classified into pregnancy-related, liver disease coincident with pregnancy or preexisting liver disease. Acute fatty liver of pregnancy (AFLP) is a rare liver disorder that is caused by defects in mitochondrial beta (β) oxidation of fatty acids. In view of its fulminant presentation and rapid progression to multiple organ failure (MOF), AFLP carries high maternal and fetal mortality. These patients are commonly present in the third trimester of pregnancy with gastrointestinal symptoms and complications such as hypoglycemia, lactic acidosis, hyperammonemia, leukocytosis, liver dysfunction, coagulopathy, and renal dysfunction. Diagnosis is mostly based on the Swansea diagnostic criteria and by excluding other etiologies of liver dysfunction. Liver biopsy is rarely performed owing to underlying coagulopathy and thrombocytopenia. In this case series, we intend to share our experience of managing four cases of AFLP that were admitted to the intensive care unit with fetal demise and MOF.