Abstract

Glucagon unresponsiveness in the fasting state characterizes a variety of hypoglycaemia with infrequent episodes usually occurring in the early morning with good health between attacks. Vomiting commonly accompanies the episodes. This variety of idiopathic hypoglycaemia has been called `ketotic hypoglycaemia', but ketonuria may be an inconsistent finding. 13 patients were compared to 8 contrast subjects with other varieties of hypoglycaemia and to 7 children without hypoglycaemia to confirm the abnormality of response to glucagon in the fasting state. 9 children were studied before and after treatment with diazoxide, 15 mg/kg per day for 3 days. 6 subjects experienced restoration of fasting glucagon response with diazoxide. The difference between responding and nonresponding subjects could either be due to variability in expression of the same metabolic defect or represent different defects in fasting energy metabolism.