Becker型肌营养不良症患者肌纤维组织化学特征的研究

Study of histochemical characteristics of muscle fiber in patients with Becker type muscular dystrophy

目的观察Becker型肌营养不良症(BMD)患者病变肌纤维组织化学特征及其临床意义。方法3例BMD患者肌肉活检标本分别进行HE染色和还原型辅酶Ⅰ四唑氮还原酶(NADHTR酶)染色,光镜下观察,并与正常对照组比较。结果BMD病例的肌肉切片镜下均可见肌纤维大小不等及显著肥大纤维、劈裂纤维、类靶子纤维、分叶状纤维、轮状纤维和环形纤维;NADHTR染色可见类靶子纤维。结论BMD病变肌纤维坏死程度轻,以变性、增生为主,其临床症状轻、病程长,是预后较好的病理学基础。

Objective To investigate the histochemical characteristics of muscle fiber in patients with Becker type muscular dystrophy （BMD） and its clinical significance. Methods Muscular biopsies were performed in 3 patients with BMD. The specimens were stained by HE and nicotinamide-adenine dinucleotid-tetrazole reducase （NADH-TR） methods and observed under light microscope. Normal muscles from healthy people were saved as controls. Results The slice from BMD patients showed varied sizes of muscular fibers, remarkable hypertrophy fibers, splitting fibers, targetoid fibers, lobulated fibers, whorled fibers and ring fibers. Targetoid fibers were also found using NADH-TR staining. Conclusions The degree of fiber necrosis in BMD is mild. Degeneration and proliferation are the main pathological changes, which predict mild clinical symptoms, long course of disease and favourable prognosis.