Ketotic hypoglycaemia of childhood

Abstract

Summary A patient with recurrent convulsions in childhood and associated ketotic hypoglycaemia is described. Hypoglycaemic attacks started at the age of 3 years and 4 months and continued until 9. At present (aged 15) the patient is mentally retarded, has epilepsy, high tone deafness and a major behaviour disturbance. Prednisone therapy failed to prevent hypoglycaemic convulsions and eventually irreversible brain damage. Intramuscular glucagon and adrenaline were ineffective in raising the blood glucose during acute hypoglycaemic attacks. Investigations at 3 years and 7 months and at 14 years showed a persistent and markedly abnormal sensitivity to a small dose of exogenous insulin with severe hypoglycaemia with convulsions, absence of clinical hyperadrenalism during hypoglycaemia, and a metabolic block in gluconeogenesis. The demonstration of a persistent biochemical abnormality of glucose metabolism at the age of 14 strongly suggests that ketotic hypoglycaemia of childhood is not another aspect of nutritional deprivation, as recently suggested (Buist, 1974), but the result of a defect in glucose homeostasis.

Review article: Accelerated starvation of childhood: Have I judged ketones?

“…These episodes have also been referred to as ‘accelerated starvation’. To distinguish the phenomenon in fasting children, it may be useful to update the nomenclature of Pierides et al from ‘Ketotic hypoglycaemia of childhood’ to ‘accelerated starvation of childhood’ (ASC). As stated earlier, rather than just a name change, this also helpfully broadens the scope to include not only children with hypoglycaemia but also a larger cohort with ketosis (or ketoacidosis) and euglycaemia who also need to be identified and managed appropriately …”

Starvation Ketosis in the Pediatric Emergency Department

“…S tarvation ketosis is a metabolic disturbance that may be present in young nondiabetic children presenting to the emergency department (ED). 1,2 Previously described as idiopathic ketotic hypoglycemia (IKH), 3 it is now known to occur in the presence of normal glucose levels, 4 and accelerated starvation of childhood (ASC) has been suggested as a better descriptor. 5 Symptoms may include lethargy, nausea, vomiting, abdominal pain, and sometimes seizures.…”