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Acute fatty liver

Up Acute fatty liver HELLP

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Charles Gomersall

First posted July 2006

Acute fatty liver of pregnancy

  • rare potentially fatal condition
  • incidence 1/6000-13000 live births

Pathogenesis

  • fetal defect in fatty acid oxidation results in accumulation of long chain fatty acids. These enter the maternal circulation and are hepatotoxic
  • long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency is a cause but not the only cause

Clinical features

  • vague abdominal pain, fatigue, nausea and vomiting
  • may present with slowly developing jaundice
  • signs of hepatic failure develop later:
    • hepatic encephalopathy
    • hypoglycaemia
  • up to 60% develop acute renal failure
  • anti-thrombin III deficiency
  • may be complicated by DIC and diabetes insipidus

Investigations

  • raised transaminases (<1000 IU/l)
  • hyperbilirubinaemia
  • prolonged PT, APTT, decreased fibrinogen
  • deranged renal function tests
  • hypoglycaemia

Differential diagnosis

  • HELLP. Marked hyperbilirubinaemia not usually a feature of HELLP
  • definitive diagnosis of acute fatty liver of pregnancy requires liver biopsy, but this is not usually necessary

Management

  • expedited delivery of fetus
  • supportive management
  • use of anti-thrombin III infusion controversial, with no clear evidence of benefit
  • may be an initial deterioration in laboratory parameters following delivery

Prognosis

  • 18% maternal mortality
  • 23% fetal mortality

Further reading

Guntupalli SR, Steingrub J. Hepatic disease and pregnancy: an overview of diagnosis and management. Crit Care Med, 2005; 33 (10, suppl): S332-9

© Charles Gomersall July 2006

©Charles Gomersall, October, 2009 unless otherwise stated. The author, editor and The Chinese University of Hong Kong take no responsibility for any adverse event resulting from the use of this webpage.
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