acute infantile liver failure
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Pathology
- transient disorder of hepatic function
Genetics
- associated with defects in TRMU
- defect in mitochondrial respiration
Clinical manifestations
- jaundice
- vomiting
- coagulopathy
- patients who survive the initial acute episode can recover & show normal development
Laboratory
- liver function tests
- serum transaminases are elevated
- serum bilirubin: hyperbilirubinemia
- coagulation studies: coagulopathy
- plasma lactate: lactic acidosis