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  • Adult Niemann-Pick disease  (1)
  • Carpal tunnel syndrome  (1)
  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 412 (1987), S. 37-45 
    ISSN: 1432-2307
    Keywords: Amyloidosis ; Carpal tunnel syndrome ; Potassium permanganate ; Immunohistochemistry ; Immunoperoxidase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary 140 biopsies from 108 patients afflicted with the carpal tunnel syndrome were studied, 27 of whom showed deposition of amyloid, in 6 of them to such an extent that the amyloid was considered significant in the pathogenesis of the carpal tunnel syndrome. Morphologically, vessels and ligaments were affected and especially the peritendinous structures. As it was always part of generalized amyloidosis, the amyloid in the carpal tunnel consisted immunohistologically of amyloid A in three cases (including one case with simultaneous amyloid deposition of the AA- and the AB-type), of amyloid Aϰ in one case, of amyloid of prealbumin origin in seventeen cases and of AB-amyloid in eight cases. We also described for the first time the manifestation of generalized senile amyloidosis (ASs) in the carpal tunnel. Deposition of amyloid ofβ-2-microglobulin type (AB) in the carpal tunnel was particularly frequent and massive.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-2307
    Keywords: Adult Niemann-Pick disease ; Generalized AL-amyloidosis ; Progressive liver failure ; Fibroblast culture ; Immunohistochemistry ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
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