Metabolic diseases that may present as acute liver failure in children

Age	Condition
Less than 6 months	Galactosemia
	Niemann-Pick type C
	Tyrosinemia
	Glycosylation defect
	Mitochondrial disease*
7 months to 4 years	Mitochondrial disease*
	Tyrosinemia
	Hereditary fructose intolerance
	Urea cycle defects
5 years to 18 years	Wilson disease
5 years to 18 years	Mitochondrial disease*

* Mitochondrial disease includes fatty acid oxidation defects, respiratory chain defects, and mitochondrial DNA depletion or damage. Mitochondrial disorders, especially Alpers-Huttenlocher disease (caused by POLG mutations), should be particularly suspected in patients presenting with valproate toxicity.

Modified with permission from: Squires RH, Alonso EM. Acute liver failure in children. In: Liver Disease in Children, 4th ed, Suchy FJ, Sokol RJ, Balistreri WF (Eds), Cambridge University Press, New York 2012. Copyright © 2012 Cambridge University Press.

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Metabolic diseases that may present as acute liver failure in children