Ketones (Ketostix, Acetest) Myoglobin Absence of reducing substances does not eliminate possibility of IEM Elevated ketones Ketones detected by Ketostix, Chemstix, Acetest Aminoacidopathies Inappropriate ketones Organic acidemias Ketonuria in neonates Carbohydrate Ketonuria, normal glucose beyond neonate intolerance disorders Low/absent ketones, hypoglycemia beyond neonate Carbohydrate production/utilization disorders Mitochondrial disorders Absent ketones, hypoketosis Fatty acid oxidation defects Myoglobin present Not always present, even with rhabdomyolysis, especially if creatinine kinase