Objective　The clinicopathological analysis of eight patients with lipid storage myopathy are presented. The pathogeny and therapeutic effect are probed into. Methods　Eight cases of lipid storage myopathy diagnosed by muscle biopsies with microscopic and electron-microscopic examination are analyzed. Quadriceps or biceps were biopsied. Muscle samples were stained with routine histology and histochemical enzyme and inspected by microscopy. Thin sections were stained with uranyl acetate followed by lead citrate prior to examination in a electron microscopy. Also, the therapeutic drugs of eight patients were evaluated. Results　Vacuole or crack of muscular fibers involved all eight patients. Sudan Black B and Oil Red O stains demonstrated increase of lipid droplets within muscle fibers. Ultrastructural examination revealed numerous lipid droplets dispersed throughout the residual myofilaments. Three cases with pathologic changed muscular fibers occupying less than 1/5 were belong to low-grade, two cases (between 1/5 to 1/3) were moderate, three cases (more than 1/2) were severe. There was one case accompanying glycogen storage disease. One case was concomitant with deficiency of cytochrome C oxidase. After prednisone treatment, seven cases had greatly improved and one case failed to respond to. Treatment using vitamin B2 together with other vitamins brought about a striking effect. Carnitine was very effective on the patients with system deficiency of carnitine. Conclusions　The pathogeny of lipid storage myopathy is varied. The confirmed diagnosis is depend on pathological features of muscle biopsy. Treatment with prednisone, carnitine, vitamins and food containing carnitine rich is very effective. It should be select the special treatment method if the pathogeny is clear.

Objective To investigate different expression levels between young and old bone marrow mesenchymal stem cells in microRNAs (miRNAs) that are significantly conserved between humans and mice.Additional studies have been conducted to discover changes in miRNA expression in old mice relative to that in young adults and discussed the roles of miRNAs in primary osteoporosis.Methods MiRNAs that are highly conserved between human and mice,and are expressed at significantly different levels in the bone marrow mesenchymal stem cells of young and old people were identified by searching the Gene Expression Omnibus (GEO) database.Human bone mesenchymal stem cells (hBMSCs) were transfected with miRNA mimics,and their relative alkaline phosphatase (ALP) activity levels were then determined.Micro-CT scanning was employed to quantitatively characterize cortical and cancellous bones of young and old mice,and to confirm that these mice accurately modeled natural aging osteoporosis.Simultaneously,we investigated differences in expression levels of miRNAs that influence ALP activity in hBMSCs in the two groups of mice.Correlations between miRNA expression levels,and parameters of bone mass and bone strength were studied.Results 28 miRNAs were found to be more than 2 fold up-regulated (down-regulated) with statistical significance (P＜0.05) in the GEO database.We also found that ALP activity was lower in hBMSCs transfected with 4 miRNAs (mir-124-3p,mir-126-3p,mir-128-3p,mir-424-5p,P＜0.05 or P＜ 0.01).The micro-CT scans indicated that the mice are accurately modeled natural aging osteoporosis.Expression of mir-124-3p increased significantly in older mice.This upregulation correlated positively with trabecular separation,and negatively with trabecular pattern factor in trabecular bone.However,in cortical bone,its expression correlated positively with trabecular separation,and negatively with bone volume fraction,trabecular number,and bone mineral density (P＜ 0.05).Conclusion Hsa-mir-124-3p,which is expressed differently in young and old bone marrow stromal cells,inhibited the osteogenic differentiation of hBMSCs.Upregulation of this miRNA in the bone tissue of aged mice may be related to the development of osteoporosis.