Ketotic hypoglycaemia is a condition occuring in young children, usually as a consequence of limitation of carbohydrate intake. Fasting of about twelve hours results in ketonaemia and ketonuria with a low blood sugar, with associated symptoms of hypoglycaemia.
The underlying defect appears to be one of regulation of alanine synthesis; alanine levels are invariably reduced. Alanine normally prevents ketogenesis by providing oxaloacetate for the tricarboxylic acid cycle (TCA). Decreased cycling of the TCA releases acetyl CoA residues from the ketogenesis pathway.
Management is with frequent high carbohydrate and protein meals.
The prognosis is excellent; children grow out of the tendency by the age of ten.
Last reviewed 01/2018