Abstract
In contrast to healthy subjects a group of children with hypoglycemic attacks do not respond with an appropriate increase in urinary epinephrine (E) during the insulin tolerance test. ITT (Broberger et al. 1959, 1961). The significance of the E deficiency regarding the disturbance of blood glucose regulation is still uncertain. Therefore, the possible importance of growth hormone (GH) and especially cortisol (F) was evaluated under various stimuli. The eight patients investigated were suffering from hypoglycemia with an insufficient or virtually absent urinary E response.
During ITT the clinical signs of hypoglycemia were lacking. The mean plasma glucose fell to a significantly lower minimum and the rise toward normalization, as expressed by the recovery index, was significantly slower. There was no impairment of GJ response.
The most impressive finding during ITT was a deficient response of plasma F which could be shown to be independently impaired from the lacking E excretion. F and E response to intravenous glucagon was also found to be impaired in the same group of patients.
The simultaneous and independent occurrence of both an adrenomedullary and adrenocortical disturbance gives evidence for an impairment of hypoglycemia-sensitive centers for E as well as F regulation located most probably in the hypothalamus. In addition, a delayed response to metopirone, a lack of sweting during hypoglycemia and a positive history for birth injury in all patients also are in favor of a central nervous system dysregulation.
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Zurbrügg, R., Tietze, H., Zuppinger, K. et al. 44. Coinbined Cortical and medullary adrenal hyporesponsiveness in hypoglycemia of infamy and Childhood. Pediatr Res 5, 92 (1971). https://doi.org/10.1203/00006450-197102000-00049
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DOI: https://doi.org/10.1203/00006450-197102000-00049