Cornstarch Therapy in Type I Glycogen-Storage Disease
- 19 January 1984
- journal article
- research article
- Published by Massachusetts Medical Society in New England Journal of Medicine
- Vol. 310 (3) , 171-175
- https://doi.org/10.1056/nejm198401193100306
Abstract
TYPE I glycogen-storage disease, an inherited absence or deficiency of glucose-6-phosphatase activity in the liver, kidney, and intestines, is associated with the accumulation of glycogen in those organs. The lack of glucose-6-phosphatase activity in the liver rapidly leads to hypoglycemia in fasting, because of the inadequate release of glucose from glucose-6-phosphate through normal glycogenolysis and gluconeogenesis. Other clinical manifestations include growth retardation, hepatomegaly, lactic acidosis, hyperuricemia, and hyperlipidemia. Most of these abnormalities have been attributed to the hypoglycemia, since treatments that maintain normal glucose concentrations correct most of the metabolic abnormalities and growth retardation. So far, the various kinds of . . .Keywords
This publication has 9 references indexed in Scilit:
- MODIFIED FOOD STARCHES FOR USE IN INFANT FOODSNutrition Reviews, 2009
- Intragastric Feeding in Type I Glycogen Storage Disease: Factors Affecting the Control of Lactic AcidemiaPediatric Research, 1981
- Development of Functional Response in Human Exocrine PancreasPediatrics, 1980
- Glucoamylase and disaccharidase activities in normal subjects and in patients with mucosal injury of the small intestineThe Journal of Pediatrics, 1980
- HYPOGLYCÆMIA COMPLICATING FEEDING REGIMENS FOR GLYCOGEN-STORAGE DISEASEThe Lancet, 1978
- Continuous Nocturnal Intragastric Feeding for Management of Type 1 Glycogen-Storage DiseaseNew England Journal of Medicine, 1976
- PORTACAVAL TRANSPOSITION IN THE TREATMENT OF GLYCOGEN-STORAGE DISEASEThe Lancet, 1966
- A NEW TEST OF PANCREATIC FUNCTION BASED ON STARCH TOLERANCEAnnals of Internal Medicine, 1954
- THE GASTRIC AND DUODENAL CONTENTS OF NORMAL INFANTS AND CHILDRENAmerican Journal of Diseases of Children, 1930