Some affected mothers and infants have inherited long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency
Carnitine palmitoyltransferase I deficiency also associated with fatty liver of pregnancy
Clinical Issues
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Most commonly occurs in last 10 weeks and usually in last 4 weeks of pregnancy
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Rapidly progressive acute liver failure
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Early diagnosis and delivery are critical
Can be fatal for mother and fetus if not diagnosed and treated early
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Treatment
Supportive care and urgent delivery of infant
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Early diagnosis and delivery associated with excellent outcome
Microscopic
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Severe microvesicular steatosis, predominantly centrilobular or diffuse
May show small rim of periportal sparing
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Zone 3 perivenular canalicular cholestasis and hepatocellular cholestasis
TERMINOLOGY
Synonyms
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Acute fatty liver of pregnancy (AFLP)
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Hepatic lipidosis of pregnancy