ACAD9 and ischemia: Although it is difficult in this case to determine the degree by which the liver dysfunction may be secondary to ischemia from circulatory collapse and cardiomyopathy, the severity of coagulopathy (INR>20), the liver histological and ultrastructural changes and the greatly reduced activity of complex I in the liver suggest that hepatocyte dysfunction linked to ACAD9 deficiency is the primary cause for ALF in this subject with multi-organ failure.