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HLA - B27:孤立性主动脉反流和严重传导系统异常的重要遗传风险因素。

HLA-B27: an important genetic risk factor for lone aortic regurgitation and severe conduction system abnormalities.

作者信息

Bergfeldt L, Insulander P, Lindblom D, Möller E, Edhag O

机构信息

Department of Medicine, Karolinska Institute, Huddinge Hospital, Stockholm, Sweden.

出版信息

Am J Med. 1988 Jul;85(1):12-8. doi: 10.1016/0002-9343(88)90497-4.

DOI:10.1016/0002-9343(88)90497-4
PMID:3260450
Abstract

PURPOSE

HLA-B27, an immunogenetic marker that is present in 8 percent of the white population around the world, has been found to be an important risk factor for the development of a group of rheumatic disorders, the seronegative spondyloarthropathies. Our objective was to assess the possible role of HLA-B27 and the associated inflammatory disease process in the development of lone aortic regurgitation.

PATIENTS AND METHODS

A group of 91 patients with lone aortic regurgitation were studied by HLA typing and clinical and roentgenologic examination.

RESULTS

The HLA-B27-associated inflammatory disease process was found to be the probable underlying cause in 15 to 20 percent of patients with lone aortic regurgitation of different degrees of severity. Furthermore, HLA-B27 was found in 88 percent of the male patients with the combination of aortic regurgitation and severe conduction system abnormalities.

CONCLUSION

We suggest that this cardiac syndrome should be regarded as an HLA-B27-associated syndrome, sometimes part of ankylosing spondylitis or Reiter's disease, but just as often presenting without obvious rheumatic disease. The marker is thus an important and widely distributed risk factor not only for the development of rheumatic disease but also for acquired aortic regurgitation and sever conduction system abnormalities.

摘要

目的

HLA - B27是一种免疫遗传标志物,在全球8%的白种人群中存在,已被发现是一组风湿性疾病——血清阴性脊柱关节病发展的重要危险因素。我们的目的是评估HLA - B27及相关炎症性疾病过程在单纯主动脉瓣关闭不全发展中的可能作用。

患者与方法

通过HLA分型以及临床和放射学检查对一组91例单纯主动脉瓣关闭不全患者进行了研究。

结果

在不同严重程度的单纯主动脉瓣关闭不全患者中,15%至20%的患者其潜在病因可能是与HLA - B27相关的炎症性疾病过程。此外,在合并主动脉瓣关闭不全和严重传导系统异常的男性患者中,88%检测到HLA - B27。

结论

我们建议,这种心脏综合征应被视为一种与HLA - B27相关的综合征,有时是强直性脊柱炎或赖特综合征的一部分,但也常常在无明显风湿性疾病的情况下出现。因此,该标志物不仅是风湿性疾病发展的重要且广泛存在的危险因素,也是后天性主动脉瓣关闭不全和严重传导系统异常的危险因素。

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