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INSULIN AND GLUCAGON SECRETION IN HEPATIC GLYCOGENOSES
Authors:SHINTARO OKADA  YOSHIKI SEINO  HIROKO KODAMA  TORU YUTAKA  KOJI INUI  MAKOTO ISHIDA  HYAKUJI YABUUCHI  YUTAKA SEINO
Abstract:ABSTRACT. Okada, S., Seino, V., Kodama, H., Yutaka, T., Inui, K., Ishida, M., Yabuuchi, H. and Seino, Y. (Department of Pediatrics, Osaka University Hospital, Fukushima-ku, Osaka and The Third Division, Department of Medicine, University of Kobe, Kobe, Japan). Insulin and glucagon secretion in hepatic glycogenoses. Acta Paediatr Scand 68: 735, 1979.—Insulin and glucagon secretion was investigated in ten patients with hepatic glycogenosis, types I and III, in order to understand the relationship between hypoglycemia and pancreatic function. In all patients, both oral glucose tolerance and intravenous arginine infusion tests revealed hypoinsulinemia. Decreased urinary C-peptide levels with standard food intake also supported hypofunction of pancreatic beta cells. On the contrary, the normal secretion pattern of glucagon in both types indicated in the arginine loading test, intact alpha cells in the pancreas. Persistent hypoinsulinism, which is apparently an adaptation to hypoglycemia, could be an important cause of nutritional dwarfism in both types of glycogenosis. The usefulness of the measurement of urinary C-peptide, which evaluates the pancreatic function and provides management for normal body growth, is discussed.
Keywords:Glycogenosis  insulin  C-peptide  glucagon
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