24例假肥大型肌营养不良症的超微结构观察  

Ultrastructural observations on 24 cases of pseudo hypertrophic muscular dystrophy

作　　者：杭振镳[1] 罗德儒[1] 吴康敏[1] 魏于全[1] 

机构地区：[1]成都华西医科大学病理学教研室,成都华西医科大学神经内科,成都华西医科大学儿科

出　　处：《中华病理学杂志》1994年第3期159-161,T031,共4页Chinese Journal of Pathology

摘　　要：用光镜及电镜观察了２４例假肥大型肌营养不良症的腓肠肌活检组织。结果显示：（１）不论是变性或是有灶性坏死的肌纤维都可以见到不同程度的质膜缺损。（２）肌纤维有一定的修复及再生能力，但此种能力极弱，不足以完全修复损伤的肌纤维。（３）病变早期肌纤维尚未明显萎缩前，已有脂肪细胞增生，因此，不能单纯用填充性作用来解释脂肪细胞的增生。用同样方法，观察了３例患儿之母及１例未出现症状的患儿之弟，其超微结构均显示出一定变化。uscular biopsy specimens of 24 cases of pseudo hypertrophic(Duchenne's)muscular dystrophy(DMD)were studied with light and electron microscopy.Ultrastructural changes include:Ⅰ.Disappear- ance of sarcolemma in focal areas of muscle fibers with degeneration or focal necrosis,which may be the preliminary muscle fiber changes in DMD. 2.Repair of sarcolemma and regenerative sarcomere were ob- served in some injured muscle fibers,but the ability to repair and regeneration being so weak that injured muscle fibers can not be completely restored.3.In the early stage of DMD when muscle fibers have not yet to become obviously atrophied, fat cell infiltration between swollen or intact muscle fibers can be ob- served but cannot be explained.In addition,3 carriers (mothers of 3 DMD patients)and one asymptomatic brother of a DMD patient were examined, some serological and ultrastructural changes were found.

关 键 词：肌营养不良 超微结构 

分 类 号：R746.202[医药卫生—神经病学与精神病学]