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目的 研究肢带型肌营养不良 (LGMD)中肌聚糖病的诊断方法。方法 在dystrophin免疫染色分型诊断基础上 ,在国内首次用α、β、γ和δ 肌聚糖的单克隆抗体对 2 5例LGMD患者肌肉标本行免疫组化和蛋白质印迹分析。结果 发现单纯γ 肌聚糖缺乏 1例 ,单纯δ 肌聚糖缺乏 2例 ,三种肌聚糖同时缺乏和四种肌聚糖同时缺乏各 1例。结论 初步诊断 5例肌聚糖病 ;各型LGMD临床表现相似 ,用四种肌聚糖的抗体对肌肉组织进行免疫组化和蛋白质印迹分析是诊断肌聚糖病的首选方法。
Objective To study the diagnosis of muscular glycanosis in limb muscular dystrophy (LGMD). Methods Based on the diagnosis of dystrophin immunostaining, the muscle samples of 25 LGMD patients were analyzed by immunohistochemistry and Western blot for the first time using monoclonal antibodies to α, β, γ and δ sarcoma in China. The results showed that only a lack of simple γ sarcoglycan in 1 case, 2 cases of lack of δ-only sarcoma, three kinds of simultaneous lack of sarcoglycan and four kinds of sarcoglycan at the same time the lack of a case. CONCLUSIONS: Five cases of initial diagnosis of muscular glycosuria were diagnosed. The clinical manifestations of each type of LGMD were similar. Immunohistochemistry and Western blot analysis of muscle tissues with four kinds of sAG antibodies were the first choice for diagnosis of muscular glycanosis.