Effect of continuous glucose therapy begun in infancy on the long-term clinical course of patients with type I glycogen storage disease

Journal of Pediatric Gastroenterology and Nutrition

Volumn 29, Issue 2, 1999, Pages 136-143

  Wolfsdorf, Joseph I ^a   Crigler Jr , John F ^a  

^a BOSTON CHILDREN S HOSPITAL   (United States)

Author keywords

Complications; Growth and development; Hypoglycemia; Metabolic control; Type I glycogenosis; Uncooked cornstarch

Indexed keywords

ALBUMIN; GLUCOSE; STARCH;

ADOLESCENT; ADULT; ANEMIA; ARTICLE; BIOCHEMISTRY; BLOOD GLUCOSE MONITORING; CHILD; CLINICAL ARTICLE; DISEASE COURSE; FEMALE; GLOMERULUS FILTRATION; GLYCOGEN STORAGE DISEASE TYPE 5; GROWTH; HEMOGLOBIN DETERMINATION; HUMAN; LIVER ADENOMA; MALE; PRIORITY JOURNAL; SEXUAL DEVELOPMENT; URINARY EXCRETION;

ADENOMA, LIVER CELL; ADOLESCENT; ADULT; ANTHROPOMETRY; BLOOD GLUCOSE; CHILD; CHILD, PRESCHOOL; FEMALE; GLUCOSE; GLYCOGEN STORAGE DISEASE TYPE I; GROWTH; HUMANS; HYPOGLYCEMIA; KIDNEY FUNCTION TESTS; LACTATES; LIVER NEOPLASMS; MALE; PUBERTY; STARCH;

GLOMERULUS;

EID: 0033508544     PISSN: 02772116     EISSN: None     Source Type: Journal    
DOI: 10.1097/00005176-199908000-00008     Document Type: Article

References (37)

1. Burchell A. Molecular pathology of glucose-6-phosphatase. FASEB J 1990;4:2978-88.
2. Chen Y-T, Burchell A. Glycogen storage diseases. In: Scriver C, Beaudet A, Sly W, Valle D, eds. The Metabolic and Molecular Bases of Inherited Disease. 7th ed. New York: McGraw-Hill; 1995:935-65.
3. Fine RN, Frasier SD, Donnell GN. Growth in glycogen-storage disease type 1: Evaluation of endocrine function. AJDC Am J Dis Child 1969;117:169-77.
4. Greene HL, Slonim AE, O'Neill JA Jr, Burr IM. Continuous nocturnal intragastric feeding for management of type 1 glycogen storage disease. N Engl J Med 1976;294:423-5.
5. Crigler JF Jr, Folkman J. Glycogen storage disease: New approaches to therapy. In: Porter R, Whelan J, eds. Hepatotrophic factors. Amsterdam: Ciba Foundation Symposium; 1977:331-51.
6. Fernandes J, Jansen hours, Jansen TC. Nocturnal gastric feeding in glucose-6-phosphatase deficient children. Pediatr Res 1979;13: 225-9.
7. Greene HL, Slonim AE, O'Neill JA Jr, Burr IM, Moran JR. Type 1 glycogen storage disease: Five years of management with nocturnal intragastric feeding. J Pediatr 1980;96:590-5.
8. Chen YT, Cornblath M, Sidbury JB. Cornstarch therapy in type I glycogen-storage disease. N Engl J Med 1984;310:171-5.
9. Wolfsdorf JI, Rudlin CR, Crigler JF Jr. Physical growth and development of children with type 1 glycogen-storage disease: Comparison of the effects of long-term use of dextrose and uncooked cornstarch. Am J Clin Nutr 1990;52:1051-7.
10. Burr IM, O'Neill JA, Karzon DT, Howard LJ, Greene HL. Comparison of the effects of total parenteral nutrition, continuous intragastric feeding, and portacaval shunt on a patient with type 1 glycogen storage disease. J Pediatr 1974;85:792-5.
11. Ehrlich R, Robinson B, Freedman M, Howard N. Nocturnal intragastric infusion of glucose in management of defective gluconeogenesis with hypoglycemia. AJDC Am J Dis Child 1978;132: 241-3.
12. Smit GPA, Berger R, Potasnick R, Moses SW, Fernandes J. The dietary treatment of children with type 1 glycogen storage disease with slow release carbohydrate. Pediatr Res 1984;18:879-81.
13. Wolfsdorf JI, Plotkin RA, Laffel LMB, Crigler JF Jr. Continuous glucose for treatment of patients with type 1 glycogen-storage disease: Comparison of the effects of dextrose and uncooked cornstarch on biochemical variables. Am J Clin Nutr 1990;52:1043-50.
14. Wolfsdorf JI, Ehrlich S, Landy HS, Crigler JF Jr. Optimal daytime feeding regimen to prevent postprandial hypoglycemia in type 1 glycogen storage disease. Am J Clin Nutr 1992;56:587-92.
15. Wolfsdorf JI, Crigler JF Jr. Cornstarch regimens for nocturnal treatment of young adults with type I glycogen storage disease. Am J Clin Nutr 1997;65:1507-11.
16. Bier DM, Leake RD, Haymond MW, et al. Measurement of "true" glucose production rates in infancy and childhood with 6,6-dideutero-glucose. Diabetes 1977;26:1016-23.
17. Greene HL, Slonim AE, Burr IM. Type 1 glycogen storage disease: a metabolic basis for advances in treatment. Adv Pediatr 1979;26: 63-92.
18. Wolfsdorf JI, Keller RJ, Landy hours, Crigler JF Jr Glucose therapy for glycogenosis type 1 in infants: comparison of intermittent uncooked cornstarch and continuous overnight glucose feedings. J Pediatr 1990;117:384-91.
19. Tanner J. Growth at adolescence. 2nd ed. Oxford, UK: Blackwell, 1962.
20. Greulich WW, Pyle SI. Radiographic atlas of skeletal development of the hand and wrist. 2nd ed. Stanford, CA: Stanford University Press, 1959.
21. Wolfsdorf J, Crigler JF Jr. Biochemical evidence for the requirement of continuous glucose therapy in young adults with type 1 glycogen storage disease. J Inher Metab Dis 1992;17:234-41.
22. Expert Panel on Blood Cholesterol Levels in Children and Adolescents. National Cholesterol Education Program: Highlights of the report of the Expert Panel on Blood Cholesterol Levels in Children and Adolescents. Pediatrics 1992;89:495-501.
23. Expert Panel on Detection, Evaluation, and Treatment of High Blood Cholesterol in Adults. Summary of the Second Report of the National Cholesterol Education Program (NCEP) Expert Panel on Detection, Evaluation, and Treatment of High Blood Cholesterol in Adults (Adult Treatment Panel II). JAMA 1993;269:3105-123.
24. Smit GPA. The long-term outcome of patients with glycogen storage disease type Ia. Eur J Pediatr 1993;152(Suppl 1):S52-S5.
25. Chen YT, Bazzarre CH, Lee MM, Sidbury JB, Coleman RA. Type I glycogen storage disease: Nine years of management with cornstarch. Eur J Pediatr 1993;152(Suppl 1):S56-9.
26. Fernandes J, Alaupovic P, Wit JM. Gastric drip feeding in patients with glycogen storage disease type 1: Its effects on growth and plasma lipids and apolipoproteins. Pediatr Res 1989;25:327-31.
27. Greene HL, Swift LL, Knapp HR. Hyperlipidemia and fatty acid composition in patients treated for type IA glycogen storage disease. J Pediatr 1991;119:398-403.
28. Ehrlich R. Nocturnal feeding for glycogen storage disease [letter]. N Engl J Med 1976;294:1125.
29. Leonard J, Dunger D. Hypoglycemia complicating feeding regimens for glycogen storage disease [letter]. Lancet 1978;2:1203-4.
30. Dunger DB, Sutton P. Hypoglycaemia complicating treatment regimens for glycogen storage disease. Arch Dis Child 1995;72: 274-5.
31. Heller S, Cryer PE. Reduced neuroendocrine and symptomatic responses to subsequent hypoglycemia after 1 episode of hypoglycemia in nondiabetic humans. Diabetes 1991;40:223-6.
32. Davis MR, Shamoon H. Counterregulatory adaptation to recurrent hypoglycemia in normal humans. J Clin Endocrinol Metab 1991; 73:995-1001.
33. Davis MR, Shamoon H. Deficient counterregulatory hormone responses during hypoglycemia in a patient with insulinoma. J Clin Endocrinol Metab 1991;72:788-92.
34. Amiel SA, Sherwin RS, Simonson DC, Tamborlane WV. Effect of intensive insulin therapy on glycemic thresholds for counterregulatory hormone release. Diabetes 1988;37:901-7.
35. Widom B, Simonson DC. Intermittent hypoglycemia impairs glucose counterregulation. Diabetes 1992;41:1597-602.
36. Fernandas J, Berger R, Smit G. Lactate as a cerebral metabolic fuel for glucose-6-phosphatase deficient children. Pediatr Res 1984;18: 335-9.
37. McCall AL. Effects of glucose deprivation on glucose metabolism in the central nervous system. In: Frier BM, Fisher BM, ed. Hypoglycaemia and diabetes: clinical and physiological aspects. London: Edward Arnold; 1993:56-71.