Studies of children with ketotic hypoglycemia.

The work of Ulstrom and his associates,"2 has described many of the features of ketotic hypoglycemia, a syndrome of sporadic hypoglycemia associated with ketosis in children. These investigators have described the following characteristics: hypoglycemia rarely occurred before 18 months of age; it usually occurred after poor dietary intake; acetonuria was present; this kind of hypoglycemia was seen in children who were small for their age and who had low birth weights, and it was associated with a depletion of hepatic glycogen stores. Most of their patients had normal intelligence and apparently outgrew the tendency to develop hypoglycemia by the time they reached adolescence. Blood sugar responses to glucose, glucagon, epinephrine, tolbutamide, leucine and ACTH were considered to be normal. However, when 14 of their 30 patients were given a ketogenic diet they developed symptomatic hypoglycemia within 24 hours, and then failed to respond to glucagon. In contrast, the control group did not become symptomatically hypoglycemic during the three days of an identical diet. On the other hand, Senior and Loridani have reported that both their patients with ketotic hypoglycemia and their control subjects became hypoglycemic in response to the ketogenic diet. Despite these studies and those of other workers,"4 the etiology and pathogenesis of this condition remain unknown and the diagnosis difficult to make. Frequently this diagnosis is made when acetonuria and hypogly-cemia coexist. However, as acetonuria has been reported in patients with several types of sporadic hypoglycemia and appears to be a nonspecific feature, its presence in the hypoglycemic child is insufficient to make the diagnosis of ketotic hypoglycemia. In order to achieve a better understanding of the etiology and pathogenesis as well as to improve the diagnostic criteria, we have undertaken a systematic evaluation of changes in blood * Associate Professor, Pediatrics.