IDIOPATHIC SPONTANEOUS HYPOGLYCEMIA IN CHILDREN

Seven cases of idiopathic spontaneous hypoglycemia in children are reported. A review is made of 51 case reports of this condition found in the literature. The onset of symptoms occurred under the age of 6 months in 60% of the cases and of these, over half showed permanent neurologic sequelae. Of 23 cases first developing symptoms when over 6 months of age only 3 later showed permanent brain damage. Of the total 58 cases, 90% first developed symptoms when under 2 years of age. Although an elevation of the blood sugar to normal levels is often seen during treatment with ACTH, some patients do not respond favorably to this treatment, nor to treatment with adrenocorticosteroids. Treatment by dietary means is of little value. Of the 58 cases, 25 underwent partial pancreatectomy; 15 were cured and 10 were not. It is the authors' belief that partial pancreatectomy should be performed in those cases which do not respond to medical treatment within 1 to 2 years. The pancreas was examined histologically in 29 cases and in 9 of these there was evidence of hypertrophy or hyperplasia of the islets of Langerhans. No familial incidence was noted with the exception of two reported series. Of the total 58 cases, 7 died; in 3, the death was a direct result of the hypoglycemia. The etiology is discussed. Although few cases have been studied, high levels of circulating insulin-like activity in serum have been previously recorded in two patients. A normal value in one patient is recorded here. Cases may be classified as leucine-sensitive and leucine-insensitive according to the blood sugar response to this amino acid. The possibility exists that another cause may be an inability to raise the blood glucose by the release of adrenalin. The "syndrome of idiopathic hypoglycemia of infants" as described by McQuarrie is believed by the authors not to be a single clinical entity.