Round Table Discussion METABOLIC DISEASES IN CHILDREN

The administration of ACTH or 11-oxygenated steroids to animals results in: (a) increased blood sugar and liver glycogen concentrations, partially as a result of decreased glucose oxidation; (b) increased urinary N excretion; (c) increased total body fat, and (d) tendency toward Na retention and K deficit. In clinical studies these findings have been largely confirmed, using indirect technics. Children on long-term treatment with full doses of adrenal hormones present a special problem, since the foregoing metabolic abnormalities cause inhibition of somatic growth. Periodic clinical appraisal of these patients with careful longitudinal measurements, body weight and x-rays for bony maturation are suggested. The importance of a food intake adequate to offset a negative N balance is stressed, as well as the provision of added K to prevent chronic K deficiency. Question: How soon after you start treatment does K deficiency develop? That is. if you are using cortisone. Dr. Gardner: Compared with desoxycorticosterone, cortisone is a relatively weak K depleting agent, and the development of clinically measurable K deficiency on full doses of cortisone may take several weeks or even longer. This variability of K depleting effect may depend to a great extent upon the dietary intake of Na, since K deficit appears to develop much more readily in the presence of adequate or more than adequate Na intake. Chairman Najjar: In bringing this discussion to an end, I would like to repeat some of the highlights presented here. We know now that some cases of hypoglycemia show absence of the alpha cells of the islet tissue of the pancreas. When pancreatic tissue is obtained under circumstances requiring surgical exploration, one must look for the alpha cells. These cells excrete a hormone, heretofore unsuspected, the hyperglycemic hormone. This is in the process of being produced commercially and should be available in the future. This hormone may well have the therapeutic potentialities for those cases, as insulin has for diabetic patients. Cases with glycogen disease may show severe and irreversible hypoglycemia. Some cases show abnormal glycogen and there is a good possibility that a defect in the enzyme system is responsible. Hormones play a significant role in carbohydrate metabolism and, when uncontrolled, may have a deleterious effect as witnessed by the development of Cushing9s syndrome following ACTH therapy.