Inflammation in dysferlin myopathy:: Immunohistochemical characterization of 13 patients
Inflammation in dysferlin myopathy:: Immunohistochemical characterization of 13 patients
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DOI：
10.1212/wnl.57.11.2136
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发表时间：
2001-12-11
期刊：
影响因子：
9.9
通讯作者：
Illa, I
中科院分区：
文献类型：
--
作者：
Gallardo, E;Rojas-García, R;Illa, I
13例不同表型的dysferlin肌病患者中有9例检测到炎症。肌内膜或血管周围浸润由11.1% +/- 6.6% C8（+）细胞、40.6% +/- 22.8% CD 4（+）细胞、36.7% +/- 23.7%巨噬细胞组成，无B细胞。主要组织相容性复合物I类在正常肌纤维中没有上调。在年轻患者中，如果有散发性近端肌无力、肌酸激酶水平非常高、肌肉活检中出现坏死纤维和炎症，则应考虑dysferlin肌病的诊断。
Inflammation was detected in 9 of 13 patients with different phenotypes of dysferlin myopathy. Endomysial or perivascular infiltrates consisted of 11.1% +/- 6.6% C8(+) cells, 40.6% +/- 22.8% CD4(+) cells, 36.7% +/- 23.7% macrophages, and no B cells. Major histocompatibility complex class I was not upregulated in normal muscle fibers. In young patients with sporadic proximal weakness, very high creatine kinase levels, necrotic fibers and inflammation in the muscle biopsy, a diagnosis of dysferlin myopathy should be considered.