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The Role of Raw Starches in the Treatment of Type I Glycogenosis

The Role of Raw Starches in the Treatment of Type I Glycogenosis Abstract The glycogenoses are a group of heritable disorders characterized by an abnormality in glycogen metabolism. The first to be described and enzymatically defined, type I glycogen storage disease (GSD), was characterized by an absence or deficiency of glucose-6-phosphatase.1 Indeed, this was the first inborn error of metabolism in which the absent or deficient enzyme was demonstrated, 52 years after Sir Archibald Garrod's prediction in 1902 that inborn errors would be found to be due to an absence of a single enzyme.2 Although there are now known to be over 15 different enzyme deficiencies associated with GSDs, the glycogenoses can be clinically divided into those that affect primarily the liver and those that affect primarily the muscle. Type I GSD is the prototype of the group that affects the liver. The associated symptoms are hypoglycemia, hepatomegaly with protuberant abdomen, enlarged kidneys, cherubic facies, and short stature. The prominent clinical References 1. Cori GT: Glycogen structure and enzyme deficiencies in glycogen storage disease. Harvey Lect 1954;45:145-171. 2. Garrod AE: The incidence of alkaptonuria: A study in chemical individuality. Lancet 1902;2:1616.Crossref 3. Starzl TE, Marckioro TL, Sexton AW, et al: The effect of portacaval transposition on carbohydrate metabolism: Experimental and clinical observations. Surgery 1965;51:687-697. 4. Greene HL, Slonim AE, O'Neill JA Jr, et al: Continuous nocturnal intragastric feeding for management of type I glycogen storage disease. N Engl J Med 1976;294:423-425.Crossref 5. Chen Y-T, Cornblath M, Sidbury JB: Cornstarch therapy in type I glycogen-storage disease. N Engl J Med 1984;310:171-175.Crossref 6. Bier DM, Leake RD, Haymmond MW, et al: Measurement of 'true' glucose production rates in infancy and childhood with 6,6-dideuteroglucose. Diabetes 1977;26:1016-1023.Crossref 7. Fernandes J, Berger R, Smit EPA: Lactate as a cerebral metabolic fuel for glucose-6-phosphatase deficient children. Pediatr Res 1954;18:335-339.Crossref 8. Dosman J, Crawhall JC, Klassen GA, et al: Urinary excretion of C6-C10 dicarboxylic acids in glycogen storage disease types I and III. Clin Chim Acta 1974;51:93-101.Crossref 9. Littlewood JM, Lec MR, Meadow SR: Treatment of Bartter's syndrome in early childhood with prostaglandin synthase inhibitors. Arch Dis Child 1978;53:43-48.Crossref 10. French D: Organization of starch granules , in Whistler RL, BeMiller JN, Paschall EF (eds): Starch, Chemistry and Technology , ed 2. New York, Academic Press Inc, 1984, pp 153-247. 11. Mobassaleh M, Montgomery RK, Biller JA, et al: Development of carbohydrate absorption in the fetus and neonate. Pediatrics 1985;75: 160-166. 12. Leonard JV, Dunger DB: Hypoglycemia complicating feeding regimens for glycogen-storage disease. Lancet 1978;2:1203-1204.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Internal Medicine American Medical Association

The Role of Raw Starches in the Treatment of Type I Glycogenosis

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References (14)

Publisher
American Medical Association
Copyright
Copyright © 1986 American Medical Association. All Rights Reserved.
ISSN
0003-9926
eISSN
1538-3679
DOI
10.1001/archinte.1986.00360140200029
Publisher site
See Article on Publisher Site

Abstract

Abstract The glycogenoses are a group of heritable disorders characterized by an abnormality in glycogen metabolism. The first to be described and enzymatically defined, type I glycogen storage disease (GSD), was characterized by an absence or deficiency of glucose-6-phosphatase.1 Indeed, this was the first inborn error of metabolism in which the absent or deficient enzyme was demonstrated, 52 years after Sir Archibald Garrod's prediction in 1902 that inborn errors would be found to be due to an absence of a single enzyme.2 Although there are now known to be over 15 different enzyme deficiencies associated with GSDs, the glycogenoses can be clinically divided into those that affect primarily the liver and those that affect primarily the muscle. Type I GSD is the prototype of the group that affects the liver. The associated symptoms are hypoglycemia, hepatomegaly with protuberant abdomen, enlarged kidneys, cherubic facies, and short stature. The prominent clinical References 1. Cori GT: Glycogen structure and enzyme deficiencies in glycogen storage disease. Harvey Lect 1954;45:145-171. 2. Garrod AE: The incidence of alkaptonuria: A study in chemical individuality. Lancet 1902;2:1616.Crossref 3. Starzl TE, Marckioro TL, Sexton AW, et al: The effect of portacaval transposition on carbohydrate metabolism: Experimental and clinical observations. Surgery 1965;51:687-697. 4. Greene HL, Slonim AE, O'Neill JA Jr, et al: Continuous nocturnal intragastric feeding for management of type I glycogen storage disease. N Engl J Med 1976;294:423-425.Crossref 5. Chen Y-T, Cornblath M, Sidbury JB: Cornstarch therapy in type I glycogen-storage disease. N Engl J Med 1984;310:171-175.Crossref 6. Bier DM, Leake RD, Haymmond MW, et al: Measurement of 'true' glucose production rates in infancy and childhood with 6,6-dideuteroglucose. Diabetes 1977;26:1016-1023.Crossref 7. Fernandes J, Berger R, Smit EPA: Lactate as a cerebral metabolic fuel for glucose-6-phosphatase deficient children. Pediatr Res 1954;18:335-339.Crossref 8. Dosman J, Crawhall JC, Klassen GA, et al: Urinary excretion of C6-C10 dicarboxylic acids in glycogen storage disease types I and III. Clin Chim Acta 1974;51:93-101.Crossref 9. Littlewood JM, Lec MR, Meadow SR: Treatment of Bartter's syndrome in early childhood with prostaglandin synthase inhibitors. Arch Dis Child 1978;53:43-48.Crossref 10. French D: Organization of starch granules , in Whistler RL, BeMiller JN, Paschall EF (eds): Starch, Chemistry and Technology , ed 2. New York, Academic Press Inc, 1984, pp 153-247. 11. Mobassaleh M, Montgomery RK, Biller JA, et al: Development of carbohydrate absorption in the fetus and neonate. Pediatrics 1985;75: 160-166. 12. Leonard JV, Dunger DB: Hypoglycemia complicating feeding regimens for glycogen-storage disease. Lancet 1978;2:1203-1204.Crossref

Journal

Archives of Internal MedicineAmerican Medical Association

Published: Feb 1, 1986

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