Acute Fatty Liver of Pregnancy

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Chapter 196 Acute Fatty Liver of Pregnancy

INTRODUCTION

Description: Acute fatty liver is a rare complication of pregnancy that results in acute liver failure, often with catastrophic consequences. It is also known as acute fatty metamorphosis or acute yellow atrophy.

Prevalence: One of 10,000 to 15,000 pregnancies.

Predominant Age: Reproductive.

Genetics: There appears to be a link to a recessively inherited mitochondrial abnormality of fatty acid oxidation, first studied in children with Reye syndrome. The most common defect is a mutation coding for long-chain3-hydroxyacyl-CoA-dehydrogenase (LCHAD).

ETIOLOGY AND PATHOGENESIS

Causes: Unknown (mimics other forms of fatty liver failure such as that induced by tetracycline in patients with impaired renal function, Reye’s syndrome, hepatotoxicity with sodium valproate, or salicylate intoxication). It is currently thought that the placenta of a fetus who is LCHAD deficient may produce a toxic metabolite that affects the maternal liver.

Risk Factors: Unknown. More common in nulliparous women, when a male fetus is present, or in multifetal gestation. The risk of recurrence in subsequent pregnancies is low, although it may be higher if the woman has a fetus who is homozygous enzyme-deficient.

CLINICAL CHARACTERISTICS

Signs and Symptoms

• Average gestational age: 37.5 weeks

• Gradual onset of malaise, anorexia, nausea and persistent vomiting, epigastric pain, and progressive jaundice

• Hypertension, proteinuria, and edema (50% of patients)

• Hypofibrinogenemia, prolonged clotting time, hyperbilirubinemia (<10 mg/dL), mild thrombocytopenia, hemolysis, markedly reduced antithrombin III levels, (severe coagulopathy in 55%), hypoglycemia.

• Elevated serum transaminase levels (300 to 500 U/L), hepatic encephalopathy (60%)

DIAGNOSTIC APPROACH

Differential Diagnosis

• Hemolysis, elevated liver enzymes, low platelet count (HELLP) syndrome

• Pre-eclampsia

• Cholestatic jaundice

• Cholelithiasis

Associated Conditions: Hypoglycemia and hepatic coma, coagulopathy, renal failure, sepsis, aspiration, circulatory collapse, pancreatitis, and gastrointestinal bleeding are all common.

Workup and Evaluation

Laboratory: Complete blood count, evaluation of liver function, serum bilirubin, clotting studies, serum ammonia.

Imaging: Ultrasonography, computed tomography, or magnetic resonance imaging may demonstrate the fatty metamorphosis, but false-negative results may be as high as 80%.

Special Tests: None indicated.

Diagnostic Procedures: History, physical and laboratory examinations.

Pathologic Findings

Grossly the liver is small, soft, yellow, and greasy. Histologically there are swollen hepatocytes with microvesicular fat and central nuclei and periportal sparing. There may also be lipid accumulation within renal tubular cells.

FOLLOW-UP

Patient Monitoring: Intensive monitoring for circulatory, renal, and hepatic collapse. Often the fetus is severely compromised (often dead at the time of diagnosis) and also requires intensive monitoring.

Prevention/Avoidance: None.

Possible Complications: At one time, this was frequently fatal for both mother (75%) and fetus (90%); lower mortality rates have been reported in recent studies (as low as 10% maternal and 20% fetal mortality). Hypoglycemia and hepatic coma (60% of patients), coagulopathy (55%), and renal failure (50%) may occur. Sepsis, aspiration, circulatory collapse, pancreatitis, and gastrointestinal bleeding are all common.

Expected Outcome: May be fatal for both mother and fetus. If the diagnosis is established and delivery is accomplished in time, recovery is marked by acute pancreatitis and ascites (almost universal). Transient diabetes insipidus is common during recovery, occurring about 7 to 10 days after delivery. For patients who receive rapid and supportive care, eventual recovery is complete and recurrence is rare.

MISCELLANEOUS

ICD-9-CM Code: 646.73.

Fesenmeier MF, Coppage KH, Lambers DS, et al. Acute fatty liver of pregnancy in 3 tertiary care centers. Am J Obstet Gynecol. 2005;192:1416.

Ibdah JA, Okajima Y, Kang XS, et al. A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women. N Engl J Med. 1999;340:1723.

Pereira SP, O’Donohue J, Wendon J, et al. Maternal and perinatal outcome in severe pregnancy-related liver disease. Hepatology. 1997;26:1258.

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.

Yang Z, Yamada J, Zhao Y, et al. Prospective screening for pediatric mitochondrial trifunctional protein defects in pregnancies complicated by liver disease. JAMA. 2002;288:2163-2166.

Barton JR, Sibai BM, Mabie WC, Shanklin DR. Recurrent acute fatty liver of pregnancy. Am J Obstet Gynecol. 1990;163:534.

Guntupalli SR, Steingrub J. Hepatic disease and pregnancy: an overview of diagnosis and management. Crit Care Med. 2005;33:S332.

Ibdah JA. Acute fatty liver of pregnancy: an update on pathogenesis and clinical implications. World J Gastroenterol. 2006;12:7397.

Kaplan MM. Acute fatty liver of pregnancy. N Engl J Med. 1985;313:367.

Ko H, Yoshida EM. Acute fatty liver of pregnancy. Can J Gastroenterol. 2006;20:25.

Rajasri AG, Srestha R, Mitchell J. Acute fatty liver of pregnancy (AFLP)—An overview. J Obstet Gynaecol. 2007;27:237.

Steingrub JS. Pregnancy-associated severe liver dysfunction. Crit Care Clin. 2004;20:763. xi.

Wolf JL. Liver disease in pregnancy. Med Clin North Am. 1996;80:1167.

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