Schematic demonstrating mitochondrial<unk>  acid beta-oxidation and effects of LCHAD deficiency, a hallmark of<unk>  The understanding of the causes of<unk>  has been improved by advances in mitochondrial biochemistry. Deficiency of LCHAD (3-hydroxyacyl-CoA dehydrogenase) leads to an accumulation of medium and long chain<unk>  acids. When this occurs in the foetus, the unmetabolized<unk>  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<unk>  is the E474Q missense mutation. LCHAD deficiency is autosomal recessive in inheritance and mothers are often found to be heterozygous for the affected mutation.
Acute fatty liver of pregnancy
Intrahepatic cholestasis of pregnancy
HELLP syndrome
Neonatal hepatitis
Puppy pregnancy syndrome
Adams–Oliver syndrome
Neonatal cholestasis
Congenital hepatic fibrosis