假肥大型肌营养不良患者骨骼肌纤维再生病理特点研究  

作　　者：赵清石[1] 鞠浩[1] 刘海燕[1] 刘川[1] 李海平[1] 

机构地区：[1]北华大学附属医院神经内科,132001

出　　处：《当代医学》2011年第3期106-107,共2页Contemporary Medicine

摘　　要：目的研究假肥大型肌营养不良患者骨骼肌纤维再生病理特点。方法收集25例假肥大型肌营养不良患者活检骨骼肌标本,其中杜兴型肌营养不良(DMD)18例,贝克型肌营养不良(BMD)7例,冷冻连续切片,采用组织化学及免疫组织化学染色,光镜下观察再生肌纤维特点,同时应用形态计量学方法分析肌纤维再生的活跃程度。结果 HE染色下见再生肌纤维深染,胞质嗜碱性,细胞核大,同源观察NCAM染色,再生肌NCAM呈阳性表达,伴随肌纤维的成熟NCAM表达逐渐减弱,成熟肌纤维无表达。DMD患者再生肌纤维直径及横截面积明显小于BMD患者再生肌纤维,再生肌纤维数量较多。结论假肥大型肌营养不良患者骨骼肌纤维再生与年龄有相关性。Objective To study pathological characteristics of skeletal muscle fibers regeneration in pseudohypertrophic muscular dystrophy patients.Methods Muscl biopsies of 25 cases of pseudohypertrophic muscular dystrophy were collected,including 18 cases of Duchenne muscular dystrophy and 7 cases of Becker muscular dystrophy.Then frozen serial sections were made and the sections were treated with HE stain and immunohistochemistry stain.The pathological characteristics of skeletal muscle fibers regeneration were observed by light microscopy,and the level of activity of regeneration fibers was analyzed by morphometry.Results Anachromasis of HE was found in the regenerated muscle fibers,basophilic cytoplasm,large nuclei,observed homologous NCAM staining fibers,Positive NCAM expression was also detected in the regenerating fibers.However,this expression taered gradually as the mature process of skeletal muscle.The expression of NCAM was negative in the maure fibers.Diameter and cross-sectional area of regeneration muscle fibers of DMD patients were significantly smaller than BMD.But quantities was larger.Conclusion The capacity of muscle fibers regeneration in pseudohypertrophic muscular dystrophy was correlated with age.

关 键 词：假肥大型肌营养不良 骨骼肌纤维 再生 病理 形态计量学 

分 类 号：R746[医药卫生—神经病学与精神病学]