Clinical characteristics of acute fatty liver of pregnancy in a tertiary Indonesian hospital

Abstract

Acute fatty liver of pregnancy (AFLP) is a rare, often autosomal recessive disorder with a major risk for maternal and perinatal mortality and morbidity. In order to achieve a more favorable outcome, awareness of its clinical signs and symptoms and early recognition are of pivotal importance. Over a 5-year period 18 patients were diagnosed with AFLP (one twin, 19 babies). The most common sign and symptoms were jaundice, hypoglycemia, nausea and vomiting, encephalopathy, and hypertension. Abnormal laboratory test results included: elevated total/ conjugated (direct) bilirubin, AST, ALT, PT, APTT, creatinine, leukocyte count, and hypoalbuminemia. Maternal and fetal mortality rate was high: 66.7% resulted in a maternal death and 57.9% in an intrauterine fetal demise (IUFD). The number of complications was found to correlate with maternal death (p = 0.042). Surviving AFLP patients had ≤ 3 complications, while patients with > 3 complications on presentation had a high risk of maternal death (OR = 5.0; 95% CI: 0.55-45.4). The presence of hypertension significantly increased the risk of maternal death (OR: 24.5; 95% CI: 1.1-542.8; p = 0.01). The risk of IUFD was primarily related to gestational age at delivery and birth weight. The high rate of jaundice as presenting symptom of AFLP suggests that Indonesian primary maternity care providers may often miss its important earlier signs and symptoms, in particular de-novo onset of nausea and vomiting in late pregnancy.