Abstract
Seven patients with glycogen disease type I have been treated with nocturnal intragastric feeding combined with frequent daytime feeding. Follow-up shows a striking improvement in their clinical condition including growth rate. Determination of biochemical parameters reveals a significant increase of lactate, pyruvate, alanine, uric acid, triglycerides, and SGOT in blood. Additional administration of an α-glucosidase inhibitor in four patients caused a significant increase in blood lactate despite unchanged blood glucose levels.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Bloom SR, Uttenthal LO, Harris A, Al-Mukhtar MYT, Yeats JC, Ghatei MA, Sagor GR, Wright NA, Polak JM (1982) Acarbose: A Model for studying intestinal adaptation and gut hormone changes in disaccharide intolerance. Gastroenterology 82:1020
Burr IM, O'Neill JA, Karzon DT, Howard LJ, Greene HL (1974) Comparison of the effects of total parenteral nutrition, continuous intragastric feeding, and portacaval shunt on a patient with Type I glucogen storage disease. J Pediat 85:792–795
Caspary WF, Graf S (1979) Inhibition of human intestinal α-glucosidehydrolases by a new complex oligosaccharide. Res Exp Med (Berl) 175:1–6
Davidson AGF, Wong LTK, Kirby L, Tze WJ, Rigg JM, Applegarth DA (1978) Glycogen storage disease Typ I: Effect of continuous nocturnal nasogastric feeding. Monogr Hum Genet, vol. 9, pp 29–36
Ehrlich RM, Robinson BH, Freedman MH, Howard NJ (1978) Nocturnal intragastric infusion of glucose in management of defective gluconeogenesis with hypoglycemia. Am J Dis Child 132:241–243
Fernandes J, Jansen H, Jansen TC (1979) Nocturnal gastric drip feeding in glucose-6-phosphatase deficient children. Pediat Res 13:225–229
Fernandes J, Koster JF, Grose WFA, Sorgedrager N (1974) Hepatic phosphorylase deficiency. Its differentiation from other hepatic glycogenoses. Arch Dis Childh 49:186–191
Greene HL, Slonim AE, O'Neill JA, Burr IM (1976) Continuous nocturnal intragastric feeding for management of type I glycogenstorage disease. N Engl J Med 294:423–425
Greene HL, Slonim AE, Burr IM, Moran JR (1980) Type I glycogen storage disease: Five years management with nocturnal intragastric feeding. J Pediat 96:590–595
Puls W, Keup U, Krause HP, Thomas G, Hoffmeister F (1977) Glucosidase inhibition. A new approach to the treatment of diabetes, obesity, and hyperlipoproteinaemia. Naturwissenschaften 64:536
Sailer D, Berg G (1979) Einflu\ eines neuen Glykosidhydrolase-Inhibitors (BAY g 5421) auf das kontinuierliche Glukogramm bei nich insulinpflichtigen Diabetikern. 14. Kongress der Deutschen Diabetes-Gesellschaft, Freiburg
Slonim AE, Lacy WW, Terry A, Greene HL, Burr IM (1979) Nocturnal intragastric therapy in type I glucogen storage disease: Effect on hormonal and amino acid metabolism. Metabolism 28:707–715
Stanley Ch A, Mills JL, Baker L (1981) Intragastric feeding in type I glycogen storage disease: Factors affecting the control of lactic acidemia. Pediatr Res 15:1504–1508
Walton RJ, Sherif IT, Noy GA, Alberti KGMM (1979) Improved metabolic profiles in insulin-treated diabetic patients given an alpha-glucosidehydrolase inhibitor. Brit Med J 1:220–221
Author information
Authors and Affiliations
Additional information
With support of the Minister für Arbeit, Gesundheit and Soziales des Landes Nordrhein-Westfalen
Rights and permissions
About this article
Cite this article
Gröbe, H., Ullrich, K. Glycogen storage disease Type I. Results of treatment with frequent daytime feeding, combined with nocturnal intragastric feeding and with administration of an α-glucosidase inhibitor. Eur J Pediatr 140, 102–104 (1983). https://doi.org/10.1007/BF00441652
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00441652