Effect of ephedrine in ketotic hypoglycaemia.

Effect of ephedrine in ketotic hypoglycaemia. The effect of oral ephedrine administration on a child with ketotic hypoglycaemia who had not responded to dietary measures alone was studied. After ephedrine administration, the hypoglycaemia developing after ketogenic stress was less profound and was substantially delayed. The impaired glycaemic response to glucagon during hypoglycaemia, characteristic of ketotic hypoglycaemia, was not altered. These observations are compatible with the proposition that ketotic hypoglycaemia is associated with impaired gluconeogenesis and they suggest that ephedrine may be a useful adjunct to therapy in this condition. The clinical features and diagnostic criteria of ketotic hypoglycaemia have been well documented (Colle and Ulstrom, 1964), but the pathophysiology and an effective therapy are still not established. Several workers reported a deficiency in epinephrine response to ketogenic stress in this condition (Koffler, Schubert, and Hug, 1971). On the basis cf this observation it has been suggested that ephedrine administration may prevent hypo-glycaemia induced by fasting or ketosis (Koffler et al., 1971). These authors reported, however, that they administered ephedrine to 3 patients without success. Rosenbloom and Tiwary (1972) reported that ephedrine sulphate improved tolerance to fasting and restored the glycaemic response to glucagon in the fasting state in 2 children they studied. In one of these children, hypoglycaemic episodes ceased during 22 months of ephedrine administration. In view of these conflicting experiences, and the failure of dietary measures alone to prevent hypo-glycaemia in some children, we have studied a child with ketotic hypoglycaemia both before and after ephedrine therapy. We wished to test the propositions that qphedrine may alter the hypoglycaemic response to ketotic stress in such children or, alternatively, that it may alter glucagon responsiveness in the presence of hypoglycaemia. because of maternal pre-eclampsia. Birthweight was 1 * 4 kg, and her condition was satisfactory until the 4th day of life when some twitching was observed. This ceased on administration of phenobarbitone. She developed normally until the age of 15 months when she had a grand mal convulsion. Another seizure occurred at 33 months associated with an upper respiratory infection, when she was found to have ketonuria and a plasma glucose value of 14 mg/100 ml. Intravenous dextrose promptly terminated the convulsion, and interval electroencephalogram was normal. Her mother was asked to test the urine regularly for the presence of ketones and was given dietary advice. Despite this, the child had recurrent seizures, all before breakfast and always associated …