Skip to main content
Log in

Glycogenosis type Ib complicated by severe granulocytopenia resembling inherited neutropenia

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

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

References

  1. Arion WJ, Wallin BK, Lange AJ, Ballas LM (1975) On the involvement of a glucose-6-phosphate transport system in the function of microsomal glucose-6-phosphatase. Mol Cell Biochem 6:75–83

    Google Scholar 

  2. Badoual J, Lestradet H, Tichet J, Sanna N, Grenet P (1972) Glycogénoses hépatiques sans déficit enzymatique reconnu se comportant cliniquement et biologiquement comme le type I de Cori. Ann Pediatr 19:507–514

    Google Scholar 

  3. Beaudet AL, Anderson DC, Arion WJ, Michels VV, Lange AJ, Hawkins EP, Stenback W (1980) Glycogenosis (GSD) Ib and abnormal neutrophil mobility with impaired glucose-6-phosphate (G6P) transport. Pediatr Res 14:519

    Google Scholar 

  4. Bialek DS, Sharp HL, Kane WJ, Elders J, Nordlie RC (1977) Latency of glucose-6-phosphatase (G-6-P) in type Ib glycogen storage disease (GSD). J Pediatr 91:838

    Google Scholar 

  5. Briggs JN, Haworth JC (1964) Liver glycogen disease: Report of a case of hyperuricemia, renal calculi and no demonstrable enzyme defect. Am J Med 36:443–449

    Google Scholar 

  6. Bührdel P, Zimmermann G, Theile H, Braun W, Beyreiss K (1977) Zur Klinik und enzymatischen Diagnostik der Glykogenose Typ I. Kinderärztl Prax 45:211–218

    Google Scholar 

  7. Chalmers RA, Ryman BE, Watts RWE (1978) Studies on a patient with in vivo evidence of type I glycogenosis and normal enzyme activities in vitro. Acta Paediatr Scand 67: 201–207

    Google Scholar 

  8. Ehrlich RM, Robinson BH, Freedman MH, Howard NJ (1978) Nocturnal intragastric infusion of glucose in management of defective gluconeogenesis with hypoglycemia. Am J Dis Child 132:241–243

    Google Scholar 

  9. L'Esperance P, Brunning R, Good A (1973) Congenital neutropenia: In vitro growth of colonies mimicking the disease. Proc Natl Acad Sci USA 70:669–674

    Google Scholar 

  10. Fernandes J, Jansen H, Jansen TC (1979) Nocturnal gastric drip feeding in glucose-6-phosphatase deficient children. Pediatr Res 13:225–229

    Google Scholar 

  11. François R, Hermier M, Ruitton-Ugliengo A (1965) Un cas de glycogénose hépatique sans déficit enzymatique reconnu. Pédiatrie 20:37–50

    Google Scholar 

  12. Greene HL, Slonim AE, Burr LM, Moran JR (1980) Type I glycogen storage disease: five years of management with nocturnal intragastric feeding. J Pediatr 96:590–595

    Google Scholar 

  13. Heyne K, Gahr M (1980) Differentiation between glycogenosis types Ia and Ib by measurement of extra respiration during phagocytosis by polymorphonuclear leukocytes? Eur J Pediatr 133:69

    Google Scholar 

  14. Howell RR (1978) The glycogen storage diseases in the metabolic basis of inherited disease. In: Stanbury CJB, Wyngaarden JB, Frederickson DS (eds) The metabolic basis of inherited disease. McGraw-Hill Book Comp, New York

    Google Scholar 

  15. Igarashi Y, Otomo H, Narisawa K, Tada K (1979) A new variant of glycogen storage disease type I: probably due to a defect in the glucose-6-phosphate transport system. J Inher Metab Dis 2:45–49

    Google Scholar 

  16. Lange AJ, Arion WJ, Beaudet AL (1980) Type Ib glycogen storage disease is caused by a defect in the glucose-6-phosphate translocase of the microsomal glucose-6-phosphatase system. J Biol Chem 255:8381–8384

    Google Scholar 

  17. McCabe ERB, Melvin TR, O'Brien D, Montgomery RR, Robinson WA, Bhasker C, Brown BI (1980) Neutropenia in a patient with type Ib glycogen storage disease: In vitro response to lithium chloride. J Pediatr 97:944–945

    Google Scholar 

  18. Narisawa K, Igarashi Y, Otomo H, Tada K (1978) A new variant of glycogen storage disease type I probably due to a defect in the glucose-6-phosphate transport system. Biochem Biophys Res Commun 83:1360–1364

    Google Scholar 

  19. Pike BL, Robinson WA (1970) Human bone marrow colony growth in agar gel. J Cell Physiol 76:77–81

    Google Scholar 

  20. Rosenfeld EL, Chibisov IV, Chistova LV, Leontjev AF, Karmansky M (1978) Two cases of unusual type I glycogenosis. Clin Chim Acta 86:295–299

    Google Scholar 

  21. Sann L, Mathieu M, Bourgeois J, Bienvenu J, Bethenod M (1980) In vivo evidence for defective activity of glucose-6-phosphatase in type Ib glycogenosis. J Pediatr 96:691–694

    Google Scholar 

  22. Schaub J, Bartholomé K, Feist D, Schmidt H (1981) Glycogenosis type Ib. Further evidence for a membrane disease. Eur J Pediatr 135:325

    Google Scholar 

  23. Senior B, Loridan L (1968) Functional differentiation of glycogenoses of the liver with respect to the use of glycerol. N Engl J Med 279:965–970

    Google Scholar 

  24. Senior B, Loridan L (1968) Studies of liver glycogenoses, with particular reference to the metabolism of intravenously administered glycerol. N Engl J Med 279:958–965

    Google Scholar 

  25. Sokol JE, Lowe CV, Sarcione EJ, Mosovich LL, Doray BH (1961) Studies of glycogen metabolism in liver glycogen disease (von Gierke's disease): six cases with similar metabolic abnormalities and responses to glucagon. J Clin Invest 40:364–374

    Google Scholar 

  26. Stegner H, Evert W, Gaedicke G (1980) Glykogenose Typ I mit normaler Aktivität der Glukose-6-Phosphatase in vitro. Monatsschr Kinderheilk 128:432–434

    Google Scholar 

  27. Wriedt K, Kaäder E, Maurer AM (1970) Defective myelopoiesis in congenital neutropenia. N Engl J Med 283:12–15

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Bartram, C.R., Przyrembel, H., Wendel, U. et al. Glycogenosis type Ib complicated by severe granulocytopenia resembling inherited neutropenia. Eur J Pediatr 137, 81–84 (1981). https://doi.org/10.1007/BF00441175

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00441175

Key words

Navigation