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  • AB-Amyloid  (1)
  • Adult Niemann-Pick disease  (1)
  • 1
    Digitale Medien
    Digitale Medien
    Springer
    Journal of molecular medicine 68 (1990), S. 1150-1155 
    ISSN: 1432-1440
    Schlagwort(e): Β 2-Mikroglobulin ; AB-Amyloid ; Dialysis ; Arthropathy ; Spondylarthropathy
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary In radioimmunological estimation ofΒ 2-microglobulin (Β 2m) significant higher serum values were found in 36 dialysis patients (44.4±20.3 mg/l) in comparison with healthy probands (1.5±0.2 mg/l). A significant relation to the duration of dialysis, diuresis, symptoms of the musculo-skeletal system, but not to radiologic changes or bone biopsy findings could be seen. Post mortem examinations carried out in 21 dialysis patients revealed AB-amyloid depositis in synovial tissue of different joints (particularly shoulder and hip joint) or intervertebral discs in eight patients (age 48 to 73 years, dialysis duration less than four years) without correlation to serumΒ 2m level or radiographically suspect areas. In the tissue of cervical spine or intervertebral discs of two patients suffering from destructive spondylarthropathy no amyloid could be detected. These results suggest that AB-amyloid may occur in elderly patients early in the course of hemodialysis and may be asymptomatic in most cases.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 2
    ISSN: 1432-2307
    Schlagwort(e): Adult Niemann-Pick disease ; Generalized AL-amyloidosis ; Progressive liver failure ; Fibroblast culture ; Immunohistochemistry ; Electron microscopy
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract We report a case in which an adult form of Niemann-Pick disease (type B of NPD) was associated with a rapidly progressive generalized AL amyloidosis of kappa type. Both diagnosis were made by biopsy, the NPD by bone marrow biopsy and fibroblast culture, the amyloidosis by liver biopsy. Malignant non-Hodgkin lymphoma was not found. The patient, a 67-year-old woman, died from hepatic coma subsequent to a progressive liver failure. We discuss possible relations between the lysosomal storage disease and the development and rapid progression of amyloidosis.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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