During a ketoacidotic crisis, intravenous fluids with glucose and electrolytes should be administered immediately. Bicarbonate (initially as 1mmol/kg over 10 minutes followed by continuous infusion) should be given to treat acidosis. Carnitine supplementation may be helpful. Dialysis is effective but usually not necessary. Unconscious patients and those with severe dyspnea may require mechanical ventilation. Long-term management involves avoidance of fasting (and intravenous glucose in cases of fever or vomiting) and, in children, a mildly restricted protein intake (1.5-2g/kg/day), avoidance of fat-rich (ketogenic) diet, and L-carnitine therapy in those with low carnitine levels. Avoidance of isoleucine overload might prevent neurological complications, but currently there is no evidence to support this{date=July 2017}}
Beta-ketothiolase deficiency
Transaldolase deficiency
Aromatic L-amino acid decarboxylase deficiency
Aldolase A deficiency
Ornithine aminotransferase deficiency
Pyruvate kinase deficiency
3-Hydroxyisobutyryl-CoA deacylase deficiency
Malonyl-CoA decarboxylase deficiency