Ketotic (idiopathic glucagon unresponsive) hypoglycaemia. Catecholamine excretion and effects of ephedrine therapy

Two children with intermittent hypoglycaemia, associated with vomiting and occurring in the morning after breakfast, were unresponsive to the hyperglycaemic effect of glucagon in the fasting state. Fed-state glucagon response was normal. Urinary catecholamine excretion rate tripled during 18 to 24 hours of fasting. Treatment with ephedrine sulphate 2·5 mg/kg body weight per day divided into 6-hourly doses, much improved fasting tolerance and restored the glycaemic response to glucagon in the fasting state. The ephedrine was without side effects and has completely eradicated hypoglycaemic episodes in one of the children during 22 months use.