European Journal of Pediatrics

, Volume 149, Issue 9, pp 630–633 | Cite as

Effects of cornstarch treatment in very young children with type I glycogen storage disease

  • M. Hayde
  • K. Widhalm
Metabolic Diseases

Abstract

Three children aged 1–2 years with glycogenosis type I were treated with 2 g/kg bodyweight oral cornstarch per meal (4–5 times a day) for a period up to 16 months. In comparison to the previous dietary regimen (day and nocturnal feedings every 3 h) the cornstarch diet stabilised serum glucose profiles and dramatically improved secondary hyperlipoproteinaemia. Mean total triglycerides decreased up to one half, consistent with a fall of very low density lipoprotein-triglycerides up to two thirds. Metabolic acidosis and hyperuricaemia did not occur and normal growth rates (0.7–1 cm/month) were achieved. We conclude that the cornstarch regimen even in the age group up to 2 years can be considered as an efficient alternative in the treatment of glycogenosis type I patients with less frequent feedings and without nocturnal infusion.

Key words

Glycogen storage disease type I Dietary management Uncooked cornstarch 

Abbreviations

GSD-I

Glycogen storage disease type I

VLDL

very low density lipoprotein

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References

  1. 1.
    Arion WJ, Wallin BK, Lange AJ, Ballas LM (1985) On the involvement of a glucose-6-phosphatase system in the function of microsomal glucose-6-phosphatase. Mol Cell Biochem 6:75–83Google Scholar
  2. 2.
    Burr IM, O'Neill JA, Karzon DT, Howard LJ, Greene ML (1974) Comparison of the effects of total parenteral nutrition, continuous intragastric feeding and portocaval shunt on a patient with type-I glycogen storage disease. J Pediatr 85:792–795PubMedGoogle Scholar
  3. 3.
    Chen YT, Cornblath M, Sidbury J (1984) Cornstarch therapy in type I glycogen storage disease. N Engl J Med 310:171–175PubMedGoogle Scholar
  4. 5.
    Cori GT (1954) Glycogen structure and enzyme deficiencies. Harvey Lect 45:145–171Google Scholar
  5. 6.
    Ehrlich LM, Robinson BH, Freedman MH, Howard NJ (1978) Nocturnal intragastric infusion of glucose in management of defective gluconeogenesis with hypoglycemia. Am J Dis Child 132:241–243PubMedGoogle Scholar
  6. 7.
    Gitzelmann R, Hirsig J (1986) Infant dumping syndrome: reversal of symptoms by feeding uncooked starch. Eur J Pediatr 145:504–506PubMedGoogle Scholar
  7. 8.
    Greene HL, Slonim AE, Burr IM, Maran JR (1980) Type I glycogen storage disease: 5 years of management with nocturnal intragastric feeding. J Pediatr 96:590–597PubMedGoogle Scholar
  8. 9.
    Howell RR, Williams JC (1983) The glycogen storage diseases. In: Stanbury JB, Wyngaarden JB, Fredrickson DS, Goldstein JL, Brown MS (eds) The metabolic basis of inherited disease, 5th edn. Mc Graw-Hill, New York. pp 141–146Google Scholar
  9. 10.
    Klumpp TG, Neale AV (1930) The gastric and duodenal contents of normal infants and children; the duodenal enzyme activity and the gastric and duodenal reactions (H-ion). Am J Dis Child 40:1215–1229Google Scholar
  10. 11.
    Lebenthal E, Lee PC (1980) Development of functional response in human exocrine pancreas. Pediatrics 66:556–560PubMedGoogle Scholar
  11. 12.
    Muller PR, Gamlen TR (1984) The activity of hepatic and lipoprotein lipase in glycogen storage disease: evidence for a circulating inhibitor of postheparin lipolytic activity. Pediatr Res 18:881–885PubMedGoogle Scholar
  12. 13.
    Schiffrin A, Polychronakos C, Abu-Srair H (1986) Glycogen storage disease type I. N Engl J Med 315:520–521PubMedGoogle Scholar
  13. 14.
    Sidbury JB, Chen YT, Roe CR (1986) The role of raw starches in the treatment of type-I glycogenosis. Arch Intern Med 146: 370–373PubMedGoogle Scholar
  14. 15.
    Smit GPA, Berger R, Potasnick R, Moses SW, Fernandes J (1984) The dietary treatment of children with type I glycogen storage disease with slow release carbohydrate. Pediatr Res 18:879–881PubMedGoogle Scholar
  15. 16.
    Smit GPA, Ververs MT, Belderok B, Van Rijn M, Berger R, Fernandes J (1988) Complex carbohydrates in the dietary management of patients with glycogenosis caused by glucose-6-phosphatase deficiency. Am J Clin Nutr 48:95–97PubMedGoogle Scholar
  16. 17.
    Wachtel U (1984) Poly- und Oligosaccharide in Säuglings- und Kindernahrungen. In: Baerlocher K, Wachtel U (eds) Bedeutung hochmolekularer Kohlenhydrate in der Säuglings- und Kinderernährung. Thieme, Stuttgart, pp 19–34Google Scholar
  17. 18.
    Williams JC (1986) Nutritional goals in glycogen storage disease. N Engl J Med 314:709–710PubMedGoogle Scholar

Copyright information

© Springer-Verlag 1990

Authors and Affiliations

  • M. Hayde
    • 1
  • K. Widhalm
    • 1
  1. 1.Department of Paediatrics, Division Metabolic DiseasesUniversity of ViennaViennaAustria

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