Acute fatty liver of pregnancy
- Richard H Lee, MD
Richard H Lee, MD
- Associate Professor of Clinical Obstetrics and Gynecology
- Division of Maternal Fetal Medicine
- Keck School of Medicine of the University of Southern California
- Tram T Tran, MD
Tram T Tran, MD
- Medical Director of Liver Transplantation
- Liver Disease and Transplant Center at Cedars-Sinai.
- Section Editors
- Keith D Lindor, MD
Keith D Lindor, MD
- Section Editor — Alcoholic and Metabolic Liver Disease
- Professor of Medicine, Mayo Clinic College of Medicine
- Dean, College of Health Solutions
- Arizona State University
- Charles J Lockwood, MD, MHCM
Charles J Lockwood, MD, MHCM
- Section Editor — Obstetrics
- Senior Vice President, USF Health
- Dean, Morsani College of Medicine
- Professor, Obstetrics and Gynecology
- University of South Florida
Acute fatty liver of pregnancy, characterized by microvesicular fatty infiltration of hepatocytes, is a disorder which is unique to human pregnancy . It was described in 1940 and was initially thought to be universally fatal . However, early diagnosis and prompt delivery have dramatically improved the prognosis, and maternal mortality should now be the exception rather than the rule .
The major clinical features of acute fatty liver of pregnancy will be reviewed here. A general approach to the pregnant woman who develops liver disease is presented elsewhere and has also been addressed in a guideline issued by the American College of Gastroenterology (table 1) . (See "Approach to liver disease occurring during pregnancy".)
Acute fatty liver of pregnancy is rare, with an approximate incidence of 1 in 7000 to 1 in 20,000 deliveries [4-8]. It is more common with multiple gestations and possibly in women who are underweight.
One of the largest population-based studies included 1,132,964 pregnancies at 229 hospitals in the United Kingdom between 2005 and 2006 . There were a total of 57 women diagnosed with acute fatty liver of pregnancy (5 cases per 100,000 pregnancies, 95% CI 3.8-6.5). Of these, 18 percent of women had twin pregnancies and 20 percent were underweight (body mass index <20).
Acute fatty liver occurs typically in the third trimester. The disease is always present before delivery, although it is not always diagnosed prior to delivery.To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Bacq Y, Riely CA. Acute fatty liver of pregnancy: the hepatologist's view. Gastroenterologist 1993; 1:257.
- Sheehan H. The pathology of acute yellow atrophy and delayed chloroform poisoning. J Obstet Gynaecol 1940; 47:49.
- Riely CA. Liver disease in the pregnant patient. American College of Gastroenterology. Am J Gastroenterol 1999; 94:1728.
- Usta IM, Barton JR, Amon EA, et al. Acute fatty liver of pregnancy: an experience in the diagnosis and management of fourteen cases. Am J Obstet Gynecol 1994; 171:1342.
- Pockros PJ, Peters RL, Reynolds TB. Idiopathic fatty liver of pregnancy: findings in ten cases. Medicine (Baltimore) 1984; 63:1.
- Reyes H, Sandoval L, Wainstein A, et al. Acute fatty liver of pregnancy: a clinical study of 12 episodes in 11 patients. Gut 1994; 35:101.
- Castro MA, Fassett MJ, Reynolds TB, et al. Reversible peripartum liver failure: a new perspective on the diagnosis, treatment, and cause of acute fatty liver of pregnancy, based on 28 consecutive cases. Am J Obstet Gynecol 1999; 181:389.
- Knight M, Nelson-Piercy C, Kurinczuk JJ, et al. A prospective national study of acute fatty liver of pregnancy in the UK. Gut 2008; 57:951.
- Riely CA. Acute fatty liver of pregnancy. Semin Liver Dis 1987; 7:47.
- Pereira SP, O'Donohue J, Wendon J, Williams R. Maternal and perinatal outcome in severe pregnancy-related liver disease. Hepatology 1997; 26:1258.
- Kennedy S, Hall PM, Seymour AE, Hague WM. Transient diabetes insipidus and acute fatty liver of pregnancy. Br J Obstet Gynaecol 1994; 101:387.
- Moldenhauer JS, O'brien JM, Barton JR, Sibai B. Acute fatty liver of pregnancy associated with pancreatitis: a life-threatening complication. Am J Obstet Gynecol 2004; 190:502.
- Castro MA, Goodwin TM, Shaw KJ, et al. Disseminated intravascular coagulation and antithrombin III depression in acute fatty liver of pregnancy. Am J Obstet Gynecol 1996; 174:211.
- Nelson DB, Yost NP, Cunningham FG. Hemostatic dysfunction with acute fatty liver of pregnancy. Obstet Gynecol 2014; 124:40.
- Grünfeld JP, Pertuiset N. Acute renal failure in pregnancy: 1987. Am J Kidney Dis 1987; 9:359.
- Schoeman MN, Batey RG, Wilcken B. Recurrent acute fatty liver of pregnancy associated with a fatty-acid oxidation defect in the offspring. Gastroenterology 1991; 100:544.
- Wilcken B, Leung KC, Hammond J, et al. Pregnancy and fetal long-chain 3-hydroxyacyl coenzyme A dehydrogenase deficiency. Lancet 1993; 341:407.
- Treem WR, Rinaldo P, Hale DE, et al. Acute fatty liver of pregnancy and long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency. Hepatology 1994; 19:339.
- Browning MF, Levy HL, Wilkins-Haug LE, et al. Fetal fatty acid oxidation defects and maternal liver disease in pregnancy. Obstet Gynecol 2006; 107:115.
- Treem WR, Shoup ME, Hale DE, et al. Acute fatty liver of pregnancy, hemolysis, elevated liver enzymes, and low platelets syndrome, and long chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency. Am J Gastroenterol 1996; 91:2293.
- Sims HF, Brackett JC, Powell CK, et al. The molecular basis of pediatric long chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with maternal acute fatty liver of pregnancy. Proc Natl Acad Sci U S A 1995; 92:841.
- Yang Z, Zhao Y, Bennett MJ, et al. Fetal genotypes and pregnancy outcomes in 35 families with mitochondrial trifunctional protein mutations. Am J Obstet Gynecol 2002; 187:715.
- Mansouri A, Fromenty B, Durand R, et al. Assessment of the prevalence of genetic metabolic defects in acute fatty liver of pregnancy (letter). J Hepatol (Denmark) 1996; 25:781.
- Bacq Y, Assor P, Gendrot C, et al. [Recurrent acute fatty liver of pregnancy]. Gastroenterol Clin Biol 2007; 31:1135.
- Ibdah JA, Bennett MJ, Rinaldo P, et al. A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women. N Engl J Med 1999; 340:1723.
- Jackson S, Kler RS, Bartlett K, et al. Combined enzyme defect of mitochondrial fatty acid oxidation. J Clin Invest 1992; 90:1219.
- Isaacs JD Jr, Sims HF, Powell CK, et al. Maternal acute fatty liver of pregnancy associated with fetal trifunctional protein deficiency: molecular characterization of a novel maternal mutant allele. Pediatr Res 1996; 40:393.
- Matern D, Hart P, Murtha AP, et al. Acute fatty liver of pregnancy associated with short-chain acyl-coenzyme A dehydrogenase deficiency. J Pediatr 2001; 138:585.
- Yang Z, Yamada J, Zhao Y, et al. Prospective screening for pediatric mitochondrial trifunctional protein defects in pregnancies complicated by liver disease. JAMA 2002; 288:2163.
- Bellig LL. Maternal acute fatty liver of pregnancy and the associated risk for long-chain 3-hydroxyacyl-coenzyme a dehydrogenase (LCHAD) deficiency in infants. Adv Neonatal Care 2004; 4:26.
- Rajasri AG, Srestha R, Mitchell J. Acute fatty liver of pregnancy (AFLP)--an overview. J Obstet Gynaecol 2007; 27:237.
- Ibdah JA. Acute fatty liver of pregnancy: an update on pathogenesis and clinical implications. World J Gastroenterol 2006; 12:7397.
- Barton JR, Sibai BM. Hepatic imaging in HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet count). Am J Obstet Gynecol 1996; 174:1820.
- Castro MA, Ouzounian JG, Colletti PM, et al. Radiologic studies in acute fatty liver of pregnancy. A review of the literature and 19 new cases. J Reprod Med 1996; 41:839.
- Campillo B, Bernuau J, Witz MO, et al. Ultrasonography in acute fatty liver of pregnancy. Ann Intern Med 1986; 105:383.
- Bacq Y. Acute fatty liver of pregnancy. Semin Perinatol 1998; 22:134.
- Riely CA, Latham PS, Romero R, Duffy TP. Acute fatty liver of pregnancy. A reassessment based on observations in nine patients. Ann Intern Med 1987; 106:703.
- Rolfes DB, Ishak KG. Acute fatty liver of pregnancy: a clinicopathologic study of 35 cases. Hepatology 1985; 5:1149.
- Nelson DB, Yost NP, Cunningham FG. Acute fatty liver of pregnancy: clinical outcomes and expected duration of recovery. Am J Obstet Gynecol 2013; 209:456.e1.
- Ockner SA, Brunt EM, Cohn SM, et al. Fulminant hepatic failure caused by acute fatty liver of pregnancy treated by orthotopic liver transplantation. Hepatology 1990; 11:59.
- Amon E, Allen SR, Petrie RH, Belew JE. Acute fatty liver of pregnancy associated with preeclampsia: management of hepatic failure with postpartum liver transplantation. Am J Perinatol 1991; 8:278.
- MacLean MA, Cameron AD, Cumming GP, et al. Recurrence of acute fatty liver of pregnancy. Br J Obstet Gynaecol 1994; 101:453.
- Barton JR, Sibai BM, Mabie WC, Shanklin DR. Recurrent acute fatty liver of pregnancy. Am J Obstet Gynecol 1990; 163:534.
- Visconti M, Manes G, Giannattasio F, Uomo G. Recurrence of acute fatty liver of pregnancy. J Clin Gastroenterol 1995; 21:243.