Skip to main content
Log in

Glycogen storage disease: recommendations for treatment

  • Review
  • Published:
European Journal of Pediatrics Aims and scope Submit manuscript

Abstract

A workshop was held on “Aspects of treatment of patients with glycogen storage disease” within the framework of the Concerted Action “Inborn errors of metabolism” of the European Communities. Consensus was reached on the main issues of treatment of patients with deficiency of glucose-6-phosphatase, glucose-6-phosphate translocase, debranching enzyme, liver phosphorylase and phosphorylase-b-kinase. The resulting recommendations are reported.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Bier DM, Leake RD, Haymond MW, Arnold KJ, Gruenke LD, Sperling MA, Kipnis DM (1977) Measurement of “true” glucose production rates in infancy and childhood with 6,6-dideuteroglucose. Diabetes 26:1016–1023

    Google Scholar 

  2. Chen Y-T, Cornblath M, Sidbury JB (1984) Cornstarch therapy in type I glycogen storage disease. N Engl J Med 310:171–175

    Google Scholar 

  3. Corbeel L, Boogaerts M, Van den Berghe G, Everaerts MC, Marchal G, Eeckels R (1983) Haematological findings in type Ib glycogen storage disease before and after portacaval shunt. Eur J Pediatr 140:273–275

    Google Scholar 

  4. Di Rocco M, Barrone B, Dallegri F, Frumento G, Patrone F (1984) Neutropenia and impaired neutrophil function in glycogenosis type Ib. J Inherited Metab Dis 7:151–154

    Google Scholar 

  5. Fernandes J (1974) The effect of disaccharides on the hyperlactacidaemia of glucose-6-phosphatase-deficient children. Acta paediatr Scand 63:695–698

    Google Scholar 

  6. Fernandes J, Van de Kamer JH (1968) Hexose and protein tolerance tests in children with liver glycogenosis caused by a deficiency of the debranching enzyme system. Pediatrics 41:935–944

    Google Scholar 

  7. Fernandes J, Pikaar NA (1969) Hyperlipemia in children with liver glycogen disease. Am J Clin Nutr 22:617–627

    Google Scholar 

  8. Fernandes J, Berger R (1987) Urinary excretion of lactate, 2-oxoglutarate, citrate and glycerol in patients with glycogenosis type I. Pediatr Res 21:279–282

    Google Scholar 

  9. Fernandes J, Berger R, Smit GPA (1984) Lactate as a cerebral metabolic fuel for glucose-6-phosphatase-deficient children. Pediatr Res 18:335–339

    Google Scholar 

  10. Greene HL, Slonim AE, O'Neill JA Jr, Burr IM (1976) Continuous nocturnal intragastric feeding for management of type I glycogen storage disease. N Engl J Med 294:423–425

    Google Scholar 

  11. Kraus J, Schlenker S, Schwedesky D (1974) Developmental changes of cerebral ketone body utilization in human infants. Hoppe-Seyler's Z Physiol Chem 355:164–170

    Google Scholar 

  12. Leonard JV, Dunger DB (1978) Hypoglycaemia complicating feeding regiments for glycogen storage disease. Lancet II:1203–1204

    Google Scholar 

  13. Moses SW, Gadoth N, Bashan N, Ben-David E, Slonim A, Wanderman KL (1986) Neuromuscular involvement in glycogen storage disease type III. Acta Paediatr Scand 75:289–296

    Google Scholar 

  14. Parker P, Burr I, Slonim A, Ghishan FK, Greene H (1981) Regression of hepatic adenomas in type la glycogen storage disease with dietary therapy. Gastroenterology 81:534–536

    Google Scholar 

  15. Schaub J, Heyne K (1983) Glycogen storage disease type Ib. Eur J Pediatr 140:283–288

    Google Scholar 

  16. Slonim AE, Coleman RA, Moses SW (1984) Myopathy and growth failure in debrancher enzyme deficiency: improvement with high-protein nocturnal enteral therapy. J Pediatr 105:906–911

    Google Scholar 

  17. Stanley CA, 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

    Google Scholar 

  18. Zangeneh F, Limbeck GA, Brown BI, Emch JR, Arcasoy MM, Goldenberg VE, Kelley VC (1969) Hepatorenal glycogenosis (type I glycogenosis) and carcinoma of the liver. J Pediatr 74:73–83

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Fernandes, J., Leonard, J.V., Moses, S.W. et al. Glycogen storage disease: recommendations for treatment. Eur J Pediatr 147, 226–228 (1988). https://doi.org/10.1007/BF00442683

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00442683

Key words

Navigation