Given the tendency for development of severe hypoglycemia during fasts, any intercurrent illness that reduces or interrupts food intake may require admission for intravenous glucose support. A skilled nutritionist should direct such changes. If the child's condition remains stable, blood studies beyond those required in routine pediatric care are not needed. Transfer is indicated when severe ketoacidosis, coma, seizures, or cerebral edema is present. Hypoglycemia and hyperammonemia may cause cerebral edema and prolonged coma.
Prognosis is difficult because of the broad clinical spectrum among affected patients.
When appropriately treated, the majority of children do well. Experience is too limited to prognosticate about life span. Genetic counseling should be provided for family members. The frequency of the homozygous state suggests the wisdom of testing for the gene in first-degree relatives of the affected child. Muscle carnitine palmityltransferase deficiency and myoglobinuria.