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  • 74.30.Ci  (1)
  • Adult Niemann-Pick disease  (1)
  • 1
    ISSN: 1432-0630
    Keywords: 74.70.Vy ; 74.30.Ci ; 74.60.Jg
    Source: Springer Online Journal Archives 1860-2000
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics , Physics
    Notes: Abstract Finely ground powders ofRBa2Cu3O7 − δ (R=Y, Nd, Sm, Eu, Dy, Ho, Er, Tm, Yb) have been mixed at dilute 3%-by-volume concentrations into epoxy matrices which were then allowed to harden in applied magnetic fieldsH A=18 kOe. X-ray diffractometry studies and 4.3 K measurements of supercurrent-induced magnetization hysteresisΔM are interpreted as indicating at least partial alignment of single-crystal-grain c-axes (1) parallel toH A forR=Y, Nd, Sm, Dy, Ho (as earlier found by Farrell et al. forR=Y), and (2) perpendicular toH A forR=Eu, Er, Tm, Yb. With a few exceptions (Y, Sm, Eu) the alignment direction correlates with the sign of the second-order Stevens factorα J of the crystalline electric field Hamiltonian in the manner suggested by Livingston et al. For the best aligned specimens (Ho, Dy) critical current densitiesJ c (4.3 K, 5 kOe) for individual grains are estimated fromΔM and the Bean model to be of order 107 A/cm2 for the measuring fieldH parallel to the original alignment fieldH A, and of order 106 A/cm2 forH perpendicular toH A.
    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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