Adrenal medullary function in idiopathic spontaneous hypoglycemia of infancy and childhood

Abstract

T HE syndrome of idiopathic spontaneously occurring hypoglycemia in infants was first described by McQuarrie in 1954 [I]. Common to the twenty-five patients studied were convulsions secondary to hypoglycemia induced by fasting. Twenty-one of the twenty-five patients were less than two years old at the onset of convulsions, eighteen were males. A family history of spontaneous hypoglycemia was present in eleven. All patients were within the normal range of height and weight for age and showed no physical signs of endocrine or metabolic disease. Spontaneous recovery occurred in most of the patients by the age of six years. McQuarrie emphasized the importance of early diagnosis in order to prevent brain damage from continued attacks of hypoglycemia. Although he was unable to demonstrate endocrine abnormalities in any of the patients, he found that the use of ACTH increased fasting blood sugar levels and reduced the frequency of convulsions. In 1959 Kinsbourne and Woolf [z] reported extensive studies on a four month old female infant with idiopathic infantile hypoglycemia. Unlike normal adults, as reported by von Euler and Luft [3], this infant did not show an increase in the urinary excretion of epinephrine in response to hypoglycemia, induced either by insulin or by withholding steroids. They could not tell whether this was due to failure to produce epinephrine or to release it into the blood stream. Each test was preceded by several days of adequate therapeutic control, excluded the possibility of exhaustion adrenal medulla by overstimulation. Broberger, Jungner and Zetterstrom, which of the