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  • 1
    Electronic Resource
    Electronic Resource
    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
    Keywords: Glycogen storage disease type Ib ; Polymorphonuclear leukocyte function ; Bactericidal activity ; Superoxide production ; Hexose monophosphate shunt
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442674
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Glycogen storage disease type Ib: familial bleeding tendency (1984)
    Springer
    European journal of pediatrics 143 (1984), S. 7-9 
    Details
    ISSN: 1432-1076
    Keywords: Glycogen storage disease type Ib ; Familial bleeding tendency ; Platelet dysfunction ; von Willebrand disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442739
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
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