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  • 1
    Digitale Medien
    Digitale Medien
    Impaired metabolic function of polymorphonuclear leukocytes in glycogen storage disease Ib (1983)
    Springer
    European journal of pediatrics 140 (1983), S. 329-330 
    Details
    ISSN: 1432-1076
    Schlagwort(e): Glycogen storage disease type Ib ; Polymorphonuclear leukocyte function ; Bactericidal activity ; Superoxide production ; Hexose monophosphate shunt
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract To elucidate the basis for the recurrent infections in patients with glycogen storage disease (GSD) Ib we tested polymorphonuclear leukocyte (PMN) function in one patient. Bactericidal capacity and phagocytosis-induced O2 consumption were reduced. Also, phorbol myristate acetate-stimulated superoxide production and glucose oxidation through the hexose monophosphate shunt were diminished compared to control subjects. Therefore it could be speculated that in PMN of patients with GSD Ib, glucose-6-phosphate has no access to the enzymes of the hexose monophosphate shunt due to a transport-related defect as shown for glucogenesis in hepatocytes.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00442674
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 2
    Digitale Medien
    Digitale Medien
    Glycogen storage disease type Ib: familial bleeding tendency (1984)
    Springer
    European journal of pediatrics 143 (1984), S. 7-9 
    Details
    ISSN: 1432-1076
    Schlagwort(e): Glycogen storage disease type Ib ; Familial bleeding tendency ; Platelet dysfunction ; von Willebrand disease
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract A mild bleeding tendency with characteristics of the von Willebrand disease was documented in family members of a girl with glycogen storage disease type Ib (GSD) Ib). It was assumed that a defective glucose-6-phosphate dependent microsomal glycoprotein synthesis was involved in the bleeding disorder of the patient and the GSD Ib heterozygotes.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00442739
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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    Artikel (Nationallizenzen)
    Volltext
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