Acute Fatty Liver of Pregnancy

Published on 30/05/2015 by admin

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Last modified 30/05/2015

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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

• Hepatitis
• Hemolysis, elevated liver enzymes, low platelet count (HELLP) syndrome
• Pre-eclampsia
• Cholestatic jaundice
• Cholelithiasis

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