This protocol is for the sick infant/child who has been previously diagnosed with 3-Methylchrotonyl-CoA (3-MCC) Dehydrogenase Deficiency (3-MCC). 3-MCC deficiency is one of several defects in the degradation pathway of leucine (a major branched-chain amino acid). Most of the defects produce metabolic ketoacidosis but ketones are absent or low despite acidosis and hypoglycemia in 3-MCC deficiency. Thus, this is a cause of hypoketotic hypoglycemia.
Click to view the complete protocol as a PDF
Disclaimer
The care of patients with metabolic disorders is complex and it is essential that a metabolic specialist be contacted as rapidly as possible. This protocol is only a guideline and should not be used for definitive treatment without metabolic consultation. Please read our Terms of Use.