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  • Arrhythmogenic right ventricular dysplasia  (1)
  • I-cell disease  (1)
  • 1
    Digitale Medien
    Digitale Medien
    Springer
    Acta neuropathologica 33 (1975), S. 285-305 
    ISSN: 1432-0533
    Schlagwort(e): I-cell disease ; Membrane-bound vacuoles ; Fibroblasts ; Mucopolysaccharidoses ; Mucopolipidoses
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract The single most characteristic morphological feature in I-cell disease (ICD) is the accumulation of membrane-bound vacuoles in mesenchymal cells (mainly fibroblasts). No true storage can be documented in those vacuoles. That their contents could have been dissolved during fixation or embedding remains however a possibility. Remnants consisting of a few lamellar arrays and of small amounts of fibrillo-granular material are too scarce for histochemical characterization. In hepatocytes large cells in the white pulp of the spleen and in myocardial fibers, vacuoles with fixative insoluble contents have been discovered; they are nowhere very abundant and their specificity is questionable. Because the affected fibroblastic elements represent a small fraction in any organ, most secondary biochemical abnormalities are expected to be detectable only in purely fibroblastic tissues. Our pathological study contributes to the understanding of some of the clinical features characteristic of ICD and stresses major morphological differences between ICD and the many diseases classified as mucopolysaccharidoses and mucolipidoses.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 2
    ISSN: 1615-2573
    Schlagwort(e): Cardiomyopathy ; Arrhythmogenic right ventricular dysplasia ; Myocarditis ; Ejection fraction ; Ventricular tachycardia
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary Twenty-four patients presenting with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ventricular tachycardia of right ventricular origin associated with structural abnormalities of the right ventricle) were divided into two groups with left ventricular ejection fraction (LVEF) above or below 45%. The distribution of LVEF in the group with LVEF below 45% was comparable with the distribution in 6 patients with idiopathic dilated cardiomyopathy who had ventricular tachycardia originating in the left ventricle (P = 0.2). They also had the same unfavorable long-term prognosis. Therefore, it is suggested that the term, arrhythmogenic right ventricular cardiomyopathy (ARVC), be restricted to patients with a LVEF below 45%. Histological data obtained in the ARVC group showed signs of acute or chronic myocarditis (in the right and left ventricles). It can be hypothesized that patients with arrhythmogenic right ventricular dysplasia (ARVD) may be prone to develop infectious myocarditis. In patients in whom an abnormal host immune response had been seen, progressive deterioration of right and left ventricular function could be observed. This pattern may be superimposed on the genetically determined background of ARVD. This could explain the wide spectrum of clinical presentation observed in patients with tachycardia originating in an abnormal right ventricle.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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