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.
- 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.