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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 149 (1990), S. 344-345 
    ISSN: 1432-1076
    Keywords: Glycogen storage disease Ib ; Secondary amyloidosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We observed the development of generalized amyloidosis in a girl with glycogen storage disease type Ib (GSD-Ib) who showed neutropenia, neutrophil dysfunction and recurrent infections. Renal and thyroid biopsies showed secondary amyloidosis, characterized by the presence of potassium permanganate sensitive Dylon positive deposits in glomeruli, renal vessels and thyroid interstitium. Immunohistochemistry showed that the deposits were composed of amyloid A (AA) protein. Possibly neutrophil abnormalities are involved in the pathogenesis of amyloidosis.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 237 (1990), S. 42-44 
    ISSN: 1432-1459
    Keywords: Acute autonomic and sensory neuropathy ; Epstein-Barr virus ; Orthostatic hypotension ; Peripheral neuropathy ; l-Threo-2,3.-dihydroxyphenylserine
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A female patient with acute autonomic and sensory neuropathy is described. Urinary disturbance developed rapidly and was followed by orthostatic syncope, absence of lacrimation, salivation and sweating, and sensory impairment. Muscle strength had been consistently normal despite diffuse muscular atrophy. Marked decrease in the number of small myelinated and unmyelinated fibres was revealed in biopsied sural nerve. Eighteen months after the onset, her autonomic symptoms have partially improved.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
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