Abstract
Corticotropin releasing factor (CRF) deficiency has not previously been demonstrated as a cause of hypoglycemic convulsions. We have recently stuided 3 children who justify such a diagnosis. One child moreover has an identical twin who provided the ideal control in the various test situations. The presence of the a disorder in ACTH release was suggested by the absence of a response in plasma cortisol and plasma ACTH to insulin induced hypoglycemia as measured by radioimmunoassay, impaired prompt water diuresis, lack of elevation in urinary 17-OHCS following metyrapone administration despite adequate 11-B-hydroxylase inhinition and normal adrenocortial activation by exogenous ACTH. Normal insuline and growth hormone responses to glucose, arginine, tolbutamide and glucagon adminstration as well as catecholamine release by insulin induced hypoglycemia confirmed the isolated nature of the defect in keeping with the presence of a normal growth pattern and normal thyroid indices. That the defeat is one of hypothalamic activation of ACTH release rather than isolated ACTH deficiency was confirmed by the release of ACTH and rise in plasma cortisol following vasopressin administration. Two children had a history of low birth weight for gestation age. The third infant was born postmaute with respiratory distress. The available evidence suggests that this is an acquired defect rather than a genetically determined abnormality. Hypoglycemia due to a defect in hypothalamic activation of ACTH release may provide the explanation for fasting hypoglycemia and convulsions in children otherwise unexplained or masquerading as ketotic hypoglycemia.
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Martin, M., Martin, A. & Calcagno, P. ENDOCRINOLOGY: Idiopathic hypoglycemia-a defect in hypothalamic ACTH-releasing factor secretion. Pediatr Res 5, 396 (1971). https://doi.org/10.1203/00006450-197108000-00106
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DOI: https://doi.org/10.1203/00006450-197108000-00106
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