Defect in Epinephrine Production in Children with Craniopharyngioma: Functional or Organic Origin?

Despite pituitary hormone replacement, patients with craniopharyngioma often complain of fatigue. They may have deficient control of catecholamine secretion caused by hypothalamic lesion. Another hypothesis is a functional defect in catecholamine production through either glucocorticoid deficiency because high intraadrenal glucocorticoid concentration is necessary for epinephrine synthesis or unrecognized hypoglycemia, which can intrinsically alter epinephrine secretion. We measured catecholamine response to insulin-induced hypoglycemia and orthostasis, and 24-h urinary catecholamine excretion, in 16 children with craniopharyngioma (patients) and 27 sex- and age-matched short children. We also studied the influence of a 4-fold increase in the usual daily dose of hydrocortisone on catecholamine excretion (50 vs. 12 mg/m2 of body surface area) in the glucocorticoid-deficient patients. Last, we compared 24-h continuous sc glucose in patients and 10 sex- and age-matched healthy children. The results are expre...