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目的 探讨骨骼肌病理活检和病变肌细胞形态定量分析对婴儿及儿童期发病脊肌萎缩症的临床诊断意义。方法 1 5岁以下脊肌萎缩症 31例 ,肌肉活检标本作常规HE染色和肌肉酶组化染色 ,应用图像分析系统对肌肉活检切片进行定量分析 ,计算每例正常细胞平均面积与病变细胞平均面积的比值。结果 肌肉病理和免疫组化改变为神经性肌萎缩 ,肌细胞变圆或呈角形 ,出现病变细胞变圆的都为 1岁以前的患者 ,而 1岁以后发病的患者大多出现呈角形细胞 ;半数有明显的同型肌群化现象。两种细胞的面积比值与发病年龄呈负相关 (r=-0 2 7,P <0 0 5) ,而与病程呈正相关 (r=0 37,P <0 0 1 ) ,与年龄没有相关关系。结论 应用图像分析系统对肌肉切片进行定量分析 ,以正常细胞与病变细胞面积比值可作为判断脊肌萎缩症病变程度的指标之一。
Objective To investigate the clinical diagnostic significance of skeletal muscle biopsy and pathological muscle cell quantitative analysis in infantile and childhood spinal muscular atrophy. Methods A total of 31 cases of spinal muscular atrophy under the age of 5 were studied. The muscle biopsy specimens were stained with HE and muscle enzyme. The image analysis system was used to quantitatively analyze the muscle biopsy sections. The average area of normal cells and the average area of diseased cells The ratio. Results Muscle pathology and immunohistochemical changes to neuromuscular atrophy, myocytes rounded or angular, appear rounded lesions were 1 year old patients, and most of the patients after 1 year of onset were angular cells; half There are obvious homogeneous muscle phenomenon. The area ratio of the two cells was negatively correlated with the age of onset (r = -0.27, P <0.05), but positively correlated with the course of disease (r = 0 37, P 0 01), but not with age . Conclusion The quantitative analysis of muscle sections with image analysis system can be used as an index to judge the severity of spinal muscular atrophy, based on the ratio of normal cells to diseased cells.