page_title: Acute fatty liver of pregnancy
section_title: Pathophysiology
hierarchical_section_title: Acute fatty liver of pregnancy / Pathophysiology
caption_attribution_description: English: Schematic demonstrating mitochondrial fatty acid beta-oxidation and effects of long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency, LCHAD deficiency Magyar: Vázlat, amely bemutatja a mitokondriális béta-oxidáció folyamatsorát, valamint a hosszú láncú 3-hydroxyacyl-koenzim-A-dehidrogenáz-hiány (LCHAD hiány) hatásait
context_page_description: Acute fatty liver of pregnancy is a rare life-threatening complication of pregnancy that occurs in the third trimester or the immediate period after delivery. It is thought to be caused by a disordered metabolism of fatty acids by mitochondria in the mother, caused by long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency. The condition was previously thought to be universally fatal, but aggressive treatment by stabilizing the mother with intravenous fluids and blood products in anticipation of early delivery has improved prognosis.
context_section_description: The understanding of the causes of acute fatty liver of pregnancy 
  has been improved by advances in mitochondrial biochemistry.  Deficiency of LCHAD (3-hydroxyacyl-CoA dehydrogenase) leads to an accumulation of medium and long chain fatty acids.  When this occurs in the foetus, the unmetabolized fatty acids will re-enter the maternal circulation through the placenta, and overwhelm the beta-oxidation enzymes of the mother.  The gene responsible for LCHAD has been isolated, and the most common mutation found in acute fatty liver of pregnancy is the E474Q missense mutation.  LCHAD deficiency is autosomal recessive in inheritance and mothers are often found to be heterozygous for the affected mutation.
caption: Schematic demonstrating mitochondrial fatty acid beta-oxidation and effects of LCHAD deficiency, a hallmark of acute fatty liver of pregnancy
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