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扩张型心肌病的形态学谱及其与免疫反应基因的关系。

The morphologic spectrum of dilated cardiomyopathy and its relation to immune-response genes.

作者信息

Arbustini E, Gavazzi A, Pozzi R, Grasso M, Pucci A, Campana C, Graziano G, Martinetti M, Cuccia M, Salvaneschi L

机构信息

Department of Pathologic Anatomy, AVIS, Pavia, Italy.

出版信息

Am J Cardiol. 1989 Nov 1;64(16):991-5. doi: 10.1016/0002-9149(89)90796-0.

DOI:10.1016/0002-9149(89)90796-0
PMID:2510489
Abstract

Endomyocardial biopsies from 174 patients with dilated cardiomyopathy (DC) were examined. Eight patients with histologically proven myocarditis were excluded from the study. A peculiar pattern of oversized and bizarre nuclei was observed in only some of the remaining patients. Two groups were identified: those with and without this feature (groups A and B, respectively). Myocyte width, nuclear diameter and nuclear/sarcoplasmic ratio were significantly higher in group A. The mean respective values were 36 +/- 5 mu, 14 +/- 3 mu and 0.41 +/- 0.08 for group A versus 20 +/- 8 mu, 7 +/- 2 mu and 0.37 +/- 0.08 for group B. Interstitial fibrosis was similarly present in groups A and B. Endocardial thickness was significantly increased in all patients, with group A showing the highest mean value. The morphologic features showed no correlation with the clinical condition of the patients at time of presentation. HLA typing was performed in 50 consecutive patients, 38 from group A and 12 from group B. DR4 and DR5 antigens were significantly more frequent in DC patients than in a normal population control (400 blood donors), while DR3 was less frequent. Group A was more strongly associated with the DR5 antigen than group B (55.3 vs 25.0%, respectively). It was less strongly associated with the DR4 antigen compared with group B (21.5 vs 41.7%, respectively). No difference was observed between the 2 groups concerning negative association with the DR3 antigen. Endomyocardial biopsies from DC patients reveal marked morphologic changes from patient to patient.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

对174例扩张型心肌病(DC)患者的心内膜心肌活检组织进行了检查。8例经组织学证实为心肌炎的患者被排除在研究之外。仅在其余部分患者中观察到一种细胞核过大且形态怪异的特殊模式。确定了两组:有此特征的患者和无此特征的患者(分别为A组和B组)。A组的心肌细胞宽度、核直径和核质比显著更高。A组的平均相应值分别为36±5μm、14±3μm和0.41±0.08,而B组分别为20±8μm、7±2μm和0.37±0.08。A组和B组均存在间质纤维化。所有患者的心内膜厚度均显著增加,A组的平均值最高。形态学特征与患者就诊时的临床状况无关。对50例连续患者进行了HLA分型,其中38例来自A组,12例来自B组。DC患者中DR4和DR5抗原的出现频率显著高于正常人群对照组(400名献血者),而DR3抗原的出现频率较低。A组与DR5抗原的关联比B组更强(分别为55.3%和25.0%)。与B组相比,A组与DR4抗原的关联较弱(分别为21.5%和41.7%)。两组在与DR3抗原的负相关方面未观察到差异。DC患者的心内膜心肌活检显示患者之间存在明显的形态学变化。(摘要截短至250字)

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