脂肪累积性肌肉病线粒体形态计量分析

目的:通过对脂肪累积性肌肉病(LSM)的肌肉标本进行线粒体形态计量分析,探讨LSM的发病机制及与线粒体肌病的关系。方法:肌活检证实的LSM30例,按照有无破碎红纤维(RRF)又分为RRF(一)的LSM-1组及RRF(+)的LSM-2组。线粒体肌病15例。正常对照15例。分别测量线粒体的平均周长、截面积、面数密度、比表面积,应用SPSS软件进行单因素方差分析及Q检验。结果:LSM-1组的线粒体各项指标与正常对照组比较无显著性差异(P>0.05)。LSM-1组与线粒体肌病组的差异有非常显著意义(P<0.01)。LSM-2组的线粒体的各项指标与正常对照组的差异有显著意义(P<0.05)。LSM-1组与LSM-2组的线粒体的各项指标的差异有显著意义(P<0.05)。结论:LSM是一病理诊断,其可由多种原因导致发病。LSM-1组可能为原发脂肪代谢异常导致。LSM-2组可能为继发于线粒体功能异常的脂肪代谢障碍,为线粒体肌病的一型。

Morphometrlc studies of muscle mitochondria in lipid storage myopathy

Objective: Morphometric studies of musele mitochondria in lipid storate myo pathy (LSM) and mitochondrial diseases were performed in order to clarify the relationship between lipid storage myopathy and mitochondrial disease. Method: 30 LSM cases confirmed by muscle biopsies were divided into 2 subgroups: without ragged-red fibers (RRF) (group 1), and with RRF (group 2) on modified Gomori trichrome staining. 15 cases of mitochondrial disease identified by muscle biopsies and 15 normal subjects were studied. Morphometric analyses on mitochondrial size, the numerical density on unit area of muscle fibers and specific surface of biopsied specimen were carried out by electron micrography. Results: A comparison of normal control and group 1 did not show significant differences in mitochondrial size, the numerical density on unit area of muscle fibers and specific surface. A comparison of group 1 and mitochondrial disease showed very significant differences in mitochondrial size, the numerical density on unit area of muscle fibers and specific surface(P<0. 01). A comparison of normal control and group 2 showed significant differences (P<0. 05). In group 2, mitochondrial size, the numerical density on unit area of muscle fibers were markedly increased, specific surface was decreased, with statistical differences observed between the group 2 and group 1(P<0. 05). Conclusions: Group 1 of LSM seems a primary disorder of lipid metabolism chief affecting skeletal muscles. Group 2 of LSM seems a secondary disorder of lipid metabolism affecting skeletal muscles which might be attributed to a kind of mitochondrial dysfunction.