Acute fatty liver of pregnancy | |
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Specialty | Obstetrics, Perinatology, Hepatology |
Complications | Death, Disseminated intravascular coagulation |
Usual onset | Third trimester of pregnancy |
Causes | Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency |
Diagnostic method | Clinical history and physical examination Liver biopsy (rarely needed) |
Treatment | Prompt delivery of the infant, Intensive supportive care Liver transplantation |
Frequency | 1 in 7,000 to 1 in 15,000 pregnancies |
Deaths | 18%[1] |
Acute fatty liver of pregnancy is a rare life-threatening complication of pregnancy that occurs in the third trimester or the immediate period after delivery.[1] It is thought to be caused by a disordered metabolism of fatty acids by mitochondria in the fetus, caused by long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency.[2] This leads to decreased metabolism of long chain fatty acids by the feto-placental unit, causing subsequent rise in hepatotoxic fatty acids in maternal plasma. The condition was previously thought to be universally fatal,[3] but aggressive treatment by stabilizing the mother with intravenous fluids and blood products in anticipation of early delivery has improved prognosis.[4]