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Fulminant hepatic failure caused by acute fatty liver of pregnancy treated by orthotopic liver transplantation

โœ Scribed by Samuel A. Ockner; Elizabeth M. Brunt; Steven M. Cohn; Elaine S. Krul; Douglas W. Hanto; Marion G. Peters


Publisher
John Wiley and Sons
Year
1990
Tongue
English
Weight
778 KB
Volume
11
Category
Article
ISSN
0270-9139

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โœฆ Synopsis


A previously healthy 35-year-old woman was seen at 37 weeks' gestation with a 10-day history of fever, vomiting, diarrhea and malaise. Serum laboratory findings included elevation of serum bilirubin and AST, prolongation of serum prothrombin time and a positive monospot. A tentative diagnosis of acute fatty liver of preg nancy was made, and a healthy male infant was delivered by emergency cesarean section because of fetal distress. Over the subsequent 3 days, acute progressive oliguric renal failure, disseminated intravascular coagulation, hypoglycemia requiring intravenous dextrose infusion and pancreatitis developed; her mental status progressed to stage I11 encephalopathy. Quantitative computed tomography estimated the liver volume to be 770 cm3. The decision to proceed with orthotopic liver transplantation was made on the basis of progressive clinical deterioration despite a g gressive support and because of her small liver size. After transplant, the patient's multisystem failure rapidly reversed. Histopathological examination of the native liver demonstrated predominantly zone 3 microvesicular steatosis with characteristic ultrastructural changes consistent with acute fatty liver of pregnancy.

Southern blot analysis for Epstein-Barr Virus DNA was negative. We conclude that orthotopic liver transplantation should be considered for the small group of patients with fulminant hepatic failure associated with acute fatty liver of pregnancy who manifest signs of irreversible liver failure despite delivery of the fetus and aggresive supportive care. (HEPATOLOGY 1990;11:59-64.) Although the mortality of acute fatty liver of pregnancy (AFLP) is reportedly declining, it remains 10% to 33% in recent clinicopathological series (1-4). This decline in mortality has been attributed to various factors, including early delivery of the infant, usually by cesarean section, earlier diagnosis and recognition of milder cases (5). This declining but persistent mortality, combined with the marked improvement in the suc-


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