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目的 研究貌似多发性肌炎的脂质沉积性肌病的肌肉病理改变 ,以解释临床现象和探讨发生机制。方法 择取脂质沉积性肌病伴血清肌酶明显升高的病人 14例 ,将临床表现、血清肌酶、肌电图和肌肉病理及肌肉组织化学改变进行总结分析。结果 临床上一般呈急性或亚急性起病 ,或呈反复发作性病程 ,肌无力明显 ,血清肌酶明显升高 ,激素治疗反应好。肌肉病理可见肌纤维内脂滴明显增多 ,形成HE染色可见的空泡 ,ORO染色证实肌纤维内有大量脂滴。其中 4例可见个别坏死肌纤维和再生纤维 ,5例有较严重的肌纤维坏死 ,对其中 3例反复肌肉活检 ,发现在脂质贮积的基础上 ,病程中的某一阶段发生明显的肌纤维坏死。其余病例虽有血清肌酶的明显升高 ,而病理上并未见到明显的肌纤维坏死 ,部分肌纤维非特异性酯酶活性增高。结论 有些脂质沉积性肌病与多发性肌炎十分相似 ,需要借助肌肉病理和肌肉组织化学检查 ,甚至反复肌肉活检才能确定。肌纤维内脂肪代谢障碍造成肌纤维破坏和肌酶的外漏 ,可能与细胞内环境改变和细胞膜通透性增加有关。
Objective To study the pathological changes of muscle in lipid-deposition myopathy which appear to be polymyositis to explain the clinical phenomenon and to explore the mechanism. Methods Fourteen patients with lipid deposition myopathy and serum creatinine were enrolled in this study. The clinical manifestations, serum creatinine, EMG, muscle pathology and chemical changes of muscle tissue were analyzed. The results were generally clinically acute or subacute onset, or was repeated episodes of disease, muscle weakness, serum creatinine was significantly elevated, hormone response was good. Muscle pathology showed a significant increase in lipid droplets within the muscle fibers, the formation of visible HE vacuoles, ORO staining confirmed a large number of lipid droplets within the muscle fibers. In 4 of them, some necrotic muscle fibers and regenerated fibers were seen, and 5 patients had more serious muscle fiber necrosis. Three of them had repeated muscle biopsies and found that there was obvious muscle fiber necrosis at a certain stage during the course of the disease on the basis of lipid storage. Although the remaining cases of serum muscle enzymes were significantly increased, while the pathology did not see significant muscle fiber necrosis, some of the non-specific muscle fiber esterase activity increased. Conclusions Some lipid-deposition myopathies are very similar to polymyositis and require muscular pathology and muscle-tissue chemistry to confirm even repeated muscle biopsies. Muscle fibers fat metabolism disorders caused by muscle fiber damage and leakage of muscle enzymes, may be related to changes in the cell environment and increased cell membrane permeability.