ABSTRACT
The classic presentation of this disease has been relatively late in infancy between 1-and 3-years-of-age, with an acute episode consistent with a diagnosis of Reye syndrome [11,13]. The episode is classic for organic acidemia in that there is massive ketosis and systemic acidosis, leading to lethargy, coma and even a fatal outcome [9]. Hypoglycemia may be prominent, symptomatic and life-threatening [6,11]. Death has also occurred from cerebral edema and cardiac arrest. There may be hyperammonemia and elevated levels of transaminases in blood. There may be microvesicular and macrovesicular deposition of fat in the liver [8,9].
