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  • 11
    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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  • 12
    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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  • 13
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 68 (1990), S. 1150-1155 
    ISSN: 1432-1440
    Keywords: Β 2-Mikroglobulin ; AB-Amyloid ; Dialysis ; Arthropathy ; Spondylarthropathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
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  • 14
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 54 (1976), S. 1011-1019 
    ISSN: 1432-1440
    Keywords: Hairy cell leukemia ; Hairy cells ; Cytochemistry ; Surface immunoglobulins ; Fc-receptors ; Phagocytosis ; Mitogen stimulation ; E-rosettes ; Haarzell-Leukämie ; Haarzellen ; Cytochemie ; Oberflächenimmunglobuline ; Fc-Rezeptoren ; Phagocytose ; Mitogen-Stimulation ; E-Rosetten
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei vier Patienten mit Haarzell-Leukämie wurden die leukämischen Zellen auf das Vorkommen von Oberflächenimmunglobulinen und Fc-Rezeptoren sowie die Stimulierbarkeit mit verschiedenen Mitogenen hin untersucht. Die Analyse dieser Eigenschaften erfolgte mit Hilfe einer kombinierten zytochemisch-radioautographischen Technik, bei der die radioaktiv markierten Zellen durch den Nachweis des Tartrat-resistenten Isoenzyms der sauren Phosphatase als Haarzellen identifiziert werden konnten. Für den Nachweis der Oberflächenimmunglobuline wurden125I-markierte (Fab')2-Fragmente von monospezifischen Antikörpern gegen schwere Immunoglobulinketten verwendet. Bei zwei Patienten wurden auf der Oberfläche der isoenzymhaltigen Zellen μ- und δ-Immunglobulinketten nachgewiesen, die bei einem großen Teil dieser Zellen gleichzeitig auf der Zelloberfläche ausgedrückt waren. Bei einem Patienten zeigten die isoenzymhaltigen Zellen γ- und δ-Ketten und bei einem vierten Patienten nur γ-Ketten. Bei einer Patientin konnten μ- und δ-Ketten nach einer in vitro-Kultur von 14 Tagen in Humanserum-freiem Medium in gleicher Weise nachgewiesen werden wie auf frischen Zellen, was für eine Synthese der Oberflächenimmunglobuline durch die Haarzellen selbst spricht. In der indirekten Immunfluoreszenztechnik wurden die Oberflächenimmunglobuline sehr schnell über einem Zellareal konzentriert, in dem auch Latexpartikel nach der Phagozytose lokalisiert waren. Mit Hilfe von125I-markierten Aggregaten von humanem IgG konnten bei allen vier Patienten Fc-Rezeptoren auf fast 100% der isoenzymhaltigen Zellen nachgewiesen werden. Eine deutliche, wenn auch geringe Stimulation der isoenzymhaltigen Zellen konnte beobachtet werden, wenn die Haarzellen mit Pokeweed-Mitogen oder Lima-Bohnen-Lektin (B- und T-Zell-Mitogene) kultiviert wurden, nicht jedoch, wenn die Stimulation mit Phytohämagglutinin oder Concanavalin-A (T-Zell-Mitogene) erfolgte. Bei keinem der drei untersuchten Patienten bildeten die Haarzellen Spontanrosetten mit Schaferythrozyten.
    Notes: Summary In four patients with hairy cell leukemia the expression of surface immunoglobulins and Fc-receptors on the leukemic cells as well as the stimulation of the leukemic cells by various T- and B-cell mitogens was studied. Using a combined cytochemical radioautographic method the tartrate resistant isoenzyme of the acid phosphatase and immunological markers could be demonstrated simultaneously on single cells. Surface immunoglobulins were detected by125I-labelled (Fab')2-fragments of monospecific anti-heavy-chain-antibodies. In two patients only μ- and δ-determinants were found on the isoenzyme-positive hairy cells; in one patient 86% and in the other 44% of these cells carried both heavy chains on the same cell. Another patient showed both γ-and δ-chains on the isoenzyme-positive cells and the fourth patient γ-chains only. When the hairy cells of one patient were cultured in vitro in human serum-free medium for 14 days, μ- and δ-determinants were found just as on freshly isolated cells suggesting that hairy cells synthesize immunoglobulins. By indirect immunofluorescence the surface-Ig on hairy cells was shown to be capped very rapidly at room temperature. The concentrated surface-Ig was found over that cytoplasmic area where most of the ingested latex particles were located. In addition, using125I-labelled aggregated human IgG (M.W. 5−15×106), Fc-receptors were found on virtually all of the isoenzyme-containing hairy cells in all four patients. Furthermore, a distinct, low-degree stimulation of the isoenzyme-positive hairy cells could be demonstrated when the cells were cultured in the presence of pokeweed mitogen or Lima-bean lectin (B- and T-cell-mitogens), whereas no stimulation was observed by phytohem-agglutinin and concanavalin A (T-cell-mitogens). In three patients studied, isoenzyme-positive hairy cells were negative with regard to rosette formation with sheep erythrocytes.
    Type of Medium: Electronic Resource
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  • 15
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Comparative Biochemistry And Physiology 33 (1970), S. 955-958+IN21-IN24+959-967 
    ISSN: 0010-406X
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 16
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Clinical Biochemistry 18 (1985), S. 201 
    ISSN: 0009-9120
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 17
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Clinical Biochemistry 18 (1985), S. 201 
    ISSN: 0009-9120
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 18
    Electronic Resource
    Electronic Resource
    Springer
    Cancer chemotherapy and pharmacology 39 (1997), S. 554-556 
    ISSN: 1432-0843
    Keywords: Key words Idarubicin ; Pharmacokinetics ; Rabbit
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  A three-compartment model was fitted to idarubicin data in a NONMEM pooled-data approach. Clearance (CL) of 221.7 ml/min was relatively high, and drug distribution was rapid (CLD=248.3 ml/min) and extensive [steady-state volume of distribution (Vss) 24 l]. The area under the concentration-time curve (AUC) of idarubicinol was 8 times that of idarubicin. Concentrations of idarubicin (idarubicinol) measured in the myocardium at 24 h after i.v. administration of idarubicin were 20 (5) times those determined in plasma. Tissue concentrations of idarubicinol were up to 400 times those of idarubicin, indicating that the active metabolite contributes significantly to the overall drug action.
    Type of Medium: Electronic Resource
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  • 19
    ISSN: 1432-0584
    Keywords: Idiopathische Amyloidose ; Immunperoxidase ; Steinmarder ; Idiopathic Amyloidosis ; Immunoperoxidase ; Marten
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Amyloid fibril proteins isolated from a spleen of a wild stone marten (Martes foina, Exleben) with idiopathic amyloidosis show resemblance to protein AA by amino acid analysis. An antiserum directed against these proteins can be used to identify the marten's amyloid in formalin-fixed tissue paraffin-embedded sections using the immunoperoxidase method.
    Notes: Zusammenfassung Amyloidfibrillen-Proteine, isoliert aus der Milz eines Steinmarders (Martes foina, Exleben) mit idiopathischer Amyloidose, zeigen Ähnlichkeit mit dem Amyloid-A-Protein. Ein Antiserum gegen diese Proteine kann die hier untersuchten Amyloid-Ablagerungen mit Hilfe der Immunperoxidase-Technik an formalin-fixierten und paraffin-eingebetteten Gewebeschnitten identifizieren.
    Type of Medium: Electronic Resource
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  • 20
    ISSN: 1432-2277
    Keywords: Key words Xenogeneic liver hemoperfusion ; Intravital microscopy ; Fucoidin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Since the main feature of hyperacute rejection is a disturbance of the xenograft's microcirculation, we analyzed microhemodynamic parameters during xenogeneic hemoperfusion of the guinea pig (GP) liver and investigated the contribution of leukocytes to the rejection process using intravital fluorescence microscopy. Isolated GP livers were hemoperfused via the portal vein in a recirculating system with a constant flow of 1 ml/min per g liver. In contrast to isogeneic perfusion with heparinized GP blood, a disturbance in the microcirculation was observed during xenogeneic perfusion using heparinized rat blood, with significantly higher values of perfusion pressure, reduced sinusoidal perfusion rates, and a larger number of stagnant leukocytes. A complete breakdown of the microcirculation, with the highest values of perfusion pressure and the smallest perfusion index, was associated with 100 % accumulated leukocytes when rat blood was anticoagulated with sodium citrate. Almost isogeneic perfusion values were obtained when fucoidin, which inhibits L-selectin-dependent cell interaction, was added to heparinized rat blood. These data indicate that leukocyte-endothelial cell interaction contributes to xenogeneic rejection.
    Type of Medium: Electronic Resource
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