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  • 1
    Electronic Resource
    Electronic Resource
    Electrophysiological and Histopathological Characteristics of Progressive Atrioventricular Block Accompanied by Familial Dilated Cardiomyopathy Caused by a Novel Mutation of Lamin A/C Gene (2005)
    350 Main Street , Malden , MA 02148-5018 , USA , and 9600 Garsington Road , Oxford OX4 2DQ , UK . : Blackwell Science Inc
    Journal of cardiovascular electrophysiology 16 (2005), S. 0 
    Details
    ISSN: 1540-8167
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Introduction: Mutations of lamin A/C gene (LMNA) cause dilated cardiomyopathy (DCM) with atrioventricular (AV) conduction defect, although the electrophysiological and histological profiles are not fully understood. Methods and Results: We analyzed a large Japanese family (21 affected and 203 unaffected members) of DCM with AV block. The responsible LMNA mutation of IVS3–10A〉G was novel and caused an aberrant splicing. The first clinical manifestation was low-grade AV block or atrial fibrillation (AF), which developed in affected members aged ≥30 years. We observed that the AV block progressed to third-degree within several years. The electrophysiological study of the four affected members revealed an impairment of intra-AV nodal conduction. Because of advanced AV block, pacemakers were implanted in 14 out of 21 affected members at the mean age of 44 years. Three affected members died suddenly and two affected members died of heart failure and/or ventricular tachycardia (VT) even after the pacemaker implantation. Postmortem examination showed conspicuous fibrofatty degeneration of the AV node. Endomyocardial biopsies showed remarkably deformed nuclei and substantial glycogen deposits in the subsarcolemma. Conclusion: The clinical phenotype in this family was characterized by (1) the first manifestation of the prolonged PQ interval or AF in adolescence, (2) progressive intra-AV nodal block to the third degree in several years, and (3) progressive heart failure after pacemaker implantation. Histological study revealed preferential degeneration at the AV node area and novel cellular damages in the working myocardium.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1540-8167.2004.40096.x
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  • 2
    Electronic Resource
    Electronic Resource
    Intestinal IgA deposition in Henoch-schönlein purpura with severe gastro-intestinal manifestations (1996)
    Springer
    European journal of pediatrics 155 (1996), S. 91-95 
    Details
    ISSN: 1432-1076
    Keywords: Biopsy ; Endoscopy ; Henoch-Schönlein purpura ; IgA ; Intestinal mucosa
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Abstract In patients with Henoch-Schönlein purpura (HSP) presenting with severe gastro-intestinal (GI) symptoms, IgA deposition was studied in endoscopically obtained mucosal biopsies. A total number of 11 patients (male, 7; female, 4) were enrolled in this study; 7 patients underwent upper GI endoscopy and biops 1 underwent sigmoidoscopy and 3 underwent both. Upper GI endoscopy in each patient showed various mucosal changes including redness, petechiae, erosions, and ulcerations, most predominant in the second part of the duodenum. Sigmoidoscopy demonstrated no abnormality in two of four patients. Intestinal deposition of IgA was positive in 7 of 11 patients with HSP. Histological examination showed non-specific inflammation of varying degrees in each patient, but no small vessel vasculitis was observed. IgA deposits were seen in only 2 of 23 control subjects with various GI diseases. Positive rate of IgA deposition per patient was significantly higher in patients with HSP than in controls (P〈0.005). Conclusion IgA deposition in the GI tract, as in the skin kidneys, is characteristic of HSP. Intestinal IgA deposition complements the diagnostic criferia of HSP.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02075757
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  • 3
    Electronic Resource
    Electronic Resource
    Intestinal IgA deposition in Henoch-Schönlein purpura with severe gastro-intestinal manifestations (1996)
    Springer
    European journal of pediatrics 155 (1996), S. 91-95 
    Details
    ISSN: 1432-1076
    Keywords: Key words Biopsy ; Endoscopy ; Henoch-Schönlein purpura ; IgA ; Intestinal mucosa
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In patients with Henoch-Schönlein purpura (HSP) presenting with severe gastro-intestinal (GI) symptoms, IgA deposition was studied in endoscopically obtained mucosal biopsies. A total number of 11 patients (male, 7; female, 4) were enrolled in this study; 7 patients underwent upper GI endoscopy and biopsy 1 underwent sigmoidoscopy and 3 underwent both. Upper GI endoscopy in each patient showed various mucosal changes including redness, petechiae, erosions, and ulcerations, most predominant in the second part of the duodenum. Sigmoidoscopy demonstrated no abnormality in two of four patients. Intestinal deposition of IgA was positive in 7 of 11 patients with HSP. Histological examination showed non-specific inflammation of varying degrees in each patient, but no small vessel vasculitis was observed. IgA deposits were seen in only 2 of 23 control subjects with various GI diseases. Positive rate of IgA deposition per patient was significantly higher in patients with HSP than in controls (P 〈 0.005). Conclusion IgA deposition in the GI tract, as in the skin and kidneys, is characteristic of HSP. Intestinal IgA deposition complements the diagnostic criteria of HSP.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310050381
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    Angiomyoma of the lower extremity: MR findings (1997)
    Springer
    Skeletal radiology 26 (1997), S. 443-445 
    Details
    ISSN: 1432-2161
    Keywords: Key words Angiomyoma ; MRI
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s002560050264
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    Paper (German National Licenses)
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