ABSTRACT
Hypoketotic hypoglycemia, metabolic acidosis, hyperammonemia; hepatomegaly; a characteristic organic aciduria: 3-hydroxy-3-methylglutaric, 3-methylglutaconic, 3-methylglutaric, and 3-hydroxyisovaleric acids; and deficiency of 3-hydroxy-3-methylglutarylCoA lyase.
