Objective To study the characteristics of sarcoglycanopathies and their diagnostic methods.Methods On basis of dystrophin immunostaining,the muscle specimens of 25 LGMD with normal dystrophin were investigated using all four sarcoglycan (?,?,?,? sarcoglycan) antibodies by immunohistochemistry and Western blotting. Results 5 specimens showed a deficiency: one case in ? SG,two in ? SG,one in three SG,and one in all four SG.Conclusion 5 patients were diagnosed as "sarcoglycanopathies". As it is difficult to distinguish the various types of LGMD based on clinical features,the immunohistochemistry and Western blotting of all four sarcoglycan antibodies may serve as an important diagnostic tool for sarcoglycanopathies.

Objective To explore the operational indications, preoperative evaluation,operative essentials and clinical prospect of endovascular repair(EVR) for aortic dissection.Methods Twenty-six patients underwent EVR for aortic dissection and all cases were Stanford B dissection.Four patients had two or more tear entries in different sites.Computed tomography angiography(CTA) or digital subtraction angiography(DSA) were used as preoperative evaluation methods.Results The grafts were installed successfully in 26 patients(100%).The patients were followed up for 1 to 18 months after treatment,and there was no endoleaks and organ or limb ischemia in all the patients.Conclusion Endovascular repair was simple,safe and effective in treating aortic dissection and dissecting aneurysm.

Objective To study the changes of neuronal nitric oxide synthase in skeletal muscles of Duchenne/Becker muscular dystrophies as to investigaing the pathogenesis of the disease. Methods NADPH diaphorase enzyme histochemistry and nNOS immunohistochemistry were used to analyze the muscle specimens from 36 patients with various muscular dystrophies and 10 normal controls. Results A positive staining was found in sarcolemma of both slow and fast-twitch muscle fibers, in 10 normal controls and 18 patients with non-muscular dystrophy as well as 12 LGMD and 2 FSHD; but a negative staining was found in 10 DMD, and a negative or faint staining in 9 BMD. The loss of nNOS in sarcolemma was associated with the loss of dystrophin, and exon 45～47 in dystrophin gene might be an important region for targeting nNOS to the sarcolemma. The deficiency of nNOS was associated with the severity of DMD/BMD, but no direct evidence for absence of nNOS in sarcolemma leading to the onset of muscle necrosis in DMD/BMD was detected.Conclusions nNOS is expressed at a high level in sarcolemma of normal muscle, and also expressed normally in muscles of those non-muscular dystrophy, LGMD and FSHD patients; but there appear absence or reduction expression in the sarcolemma of DMD and BMD. nNOS is involved in regulating of physiological functions of skeletal muscles and may be an important role in pathogenesis of DMD/BMD.

Objective To develop and compare the methods for determining the carrier status in the 18 deleted families of Duchenne and Becker muscular dystrophy. Methods Deletion analysis of the probands was performed by multiplex polymerase chain reaction (PCR) to amplify 9 dystrophin exons described by Chamberlain. Polymorphism linkage analysis was made on DNA with PCR amplification using primers of intragenic short tandem repeat sequences (STR44, STR45, STR49 and STR50), primers of 5′ end (5′DYS II) and primers of 3′end (MZ18, MZ19) in the members of the families. Gene dosage analysis was performed and DQ value was calculated. Results Both of deletions of exons and STR allelic fragments adjacent to the deleted exons were determined in the probands. STR allelic fragments of 6 pairs of heterozygotes, 2 pairs of homozygotes and 11 hemizygotes were detected at those loci in all of the female relatives. 13 female relatives in deleted families were assayed with gene dosage analysis. In 9 /13 female relatives DQ value was in the range of single copy and carrier status was ascertained.Conclusion Repeat sequence polymorphism as well as gene dosage analysis can potentially be used in carrier detection in the deleted families of Duchenne and Becker muscular dystrophy.

Tetrandrine (Tet) is a bibenzylisoquinoline alkaloid isolated from Stephania tetrandra S Morr. Lots of studies demonstrated that Tet could: ① act as a calcium antagonist via blocking plasma membrane voltage- or receptor-operating calcium channels, inhibiting extracellular calcium entry and intracellular calcium mobilization, so it could prevent hepatocytes, cardiomyocytes, pancreas cells and neurocytes from toxic or ischemia-reperfusion injuries. However, in HL-60 and leukamia T cells, Tet promoted calcium releasing from mitochondria or/and microsomes and induced these cells death. ② down-regulate T cell protein kinase C signal transduction pathway, inhibit T cell proliferation, interleukin-2 secretion and expression of the T cell activation antigen. It could also interrupted integrity of macrophages, reduced neutrophiles and macrophages respiratory-bursting and proinflammatory cytokines secretion through minimizing nuclear transcription factor kappa B DNA binding activity. ③ induce tumor cells apoptosis. ④ down-regulate P glucoprotein activity and reverse tumor cells multidrug resistance. ⑤ also inhibit platelet-derived growth factor induced hepaticstellate cells and human lung fibroblast proliferation, down-regulete type Ⅰ and type Ⅲ collagen secretion. In this article, we also reviewed the therapeutic effects of Tet on hepatic fibrosis, pulmonary fibrosis, portal hypertension, pulmonary hypertension, anti-inflammation and anti-tumors.

Objective Activin A, a member of the transforming growth factor (TGF ?) superfamily, has been reported to overexpress in liver cirrhosis. The aim of this study was to examine the expression changes of activin A in the process of carbon tetrachloride induced rat hepatic fibrosis. Methods Hepatic fibrosis was induced in rats by subcutaneous injections of 40% carbon tetrachloride oily solution for a period of 1 to 7 weeks. 6～10 rats were killed at week 1,2,3,4,5,6 and 7 weeks. The kinetics of activin A messenger RNA expression and its protein localization were assayed by semi quantity reverse transcription polymerase chain reaction (RT PCR) and immunohistochemistry. Results RT PCR and immunohistochemistry showed normal rat liver could express activin A mRNA and protein, and its expression was transiently decreased and became undectable after carbon tetrachloride injections for 2 or 3 weeks, then increased grandually. After carbon tetrachloride injecting for 6 and 7 weeks, activin A mRNA and protein expression were significantly enhanced in rats liver ( P

Objective To investigate the effects of different doses of angiotensin Ⅱ(Ang Ⅱ) on hepa tic stellate cells(HSC) proliferation and collagen synthesis in rats. Methods The pronase E and collagen Ⅰ were used to isolate HSC, 3H TdR, 3H Leu and 3H Pro incorporation methods were used to evaluate the effects of different doses of AngⅡ on HSC DNA, protein and proline synthesis. Results It showed that 10 -9 ～10 -6 mol/L AngⅡ could significantly increase the 3H TdR incorporation rate of HSC ( P

Objective To explore the effects of urokinase type plasminogen activator (uPA) gene-modified bone marrow-derived liver stem cells ( BDLSC) transplantation on hepatocyte regeneration in CCl4-induced liver fibrosis rats. Methods Ten male Fisher 344 rats were donor rats of BDLSC. The BDLSC of male rat was transfected with AduPA. Thirty-six female Fisher 344 rats were equally divided into normal group (injected subcutaneously with olive oil) , model group (CCl4 induced the model, injected through tail vein with 0. 9% sodium chloride), BDLSC group (CCl4 induced the model, injected through tail vein with BDLSC) and gene transfected group (CCl4 induced the model,injected through tail vein with gene transfected BDLSC). Liver function and area of collagen were observed. The expression of hepatic growth factor ( HGF) and its receptor c-met mRNA in rats' liver tissues were tested by semiquantitative RT-PCR. The expressions of proliferating cell nuclear antigen (PCNA) in rats' liver tissues were determined by immunohistochemistry staining. Results The areas of collagen in normal group, model group, BDLSC group and gene transfected group was 0. 12% ± 0.03%, 14. 49%±1.40%, 8. 25%±0. 82% and 5. 12%±0. 40% accordingly, there were significant differences between groups (P<0. 05). Compared with model group and BDLSC group, the liver function of gene transfected group significantly improved, the serum levels of hyaluronic acid (HA),procollagen Ⅲ (PCⅢ) and the content of hydroxyproline in liver tissues decreased dramatically. The expression of HGF and c-met at mRNA levels were up-regulated significantly, and the expression of PCNA protein in liver tissues increased obviously. Conclusion uPA gene-modified BDLSC transplantation may induce proliferation of hepatocytes, and then improve the liver functions of fibrotic rats induced by CCl4.

Objective To investigate the clinical effect of early application of nimodipine on a large area cerebral infarction after severe traumatic brain injury operation.Methods Fifty-one patients with severe head injury after large area cerebral infarction were as treatment group who hospitalized from January 2009 to January 2012,and 48 hospitalized cases as the control group from January 2005 to January 2008.The patients in control group were received drugs to decrease intracranial pressure,and enhance nerve nutrition therapy,while in the treatment group,beside the therapy method of control group,were received nimodipine intravenously by micro-pump for 10 days,and then oral administration for 10 days.Plasma endothelin-1 was detected at 0,5th,7th,14th day days after hospitalization.Dopple was pplied to record the middle cerebral artery (MCA) peak systolic velocity(Vp) of the injured side for 7 d.Glasgow outcome score(GOS) was recorded in the 3 months follow-up.The awakening time was recorded consciousness.Results At 21 st day after treatment,22 cases were died in the treatment group and survival patients with cerebral vasospasm were 14 cases (48.28％,14/29).However,30 cases were died in control group and cerebral vasospasm(CVS) of survival patients was 15 cases (83.33％,15/18),significantly higher than that in treatment group (x2 =5.78,P ＜ 0.05).The variable tendencies of Vp,plasma endothelin-1 and the intracranial pressure were significantly different between the treatment group and the control group (Vp:F group =276.27,Ftime =603.54,F interactive =85.68 ; plasma endothelin-1:F grouP =281.16,F time =608.32,F interactive =87.45 ; intracranial pressure:F grouP =326.58,F time =78.63,F interactive =27.39 ; P ＜ 0.05).Mter 3 months of treatment,the value of GOS was significandy higher in treatment group than that of control group (x2 =4.76,P ＜ 0.05).Furthermore through three months treatment,the effective rate in treatment group was higher than that in the control group (52.94％ (27/51) vs.(31.25％ (15/48)),the awakening periods was shorter than that in control group((20.7 ±6.5) d vs.(27.8 ± 7.6) d,t =3.19,P ＜ 0.05)).Conclusion Early applications of nimodipine treatment after severe traumatic brain injury patients with massive cerebral infarction can significantly improve the clinical efficacy and shorten the duration of coma.

AIM: To investigate the effects of octreotide (Oct) on the proliferation and extracellular matrix (ECM) synthesis in hepatic stellate cells (HSCs). METHODS: HSCs were isolated from normal male Sprague-Dawley rat liver by a combination of pronase-collagenase perfusion and density gradient centrifugation. The concentration of 2.5 ?g/L transforming growth factor ?1 (TGF?1) was used in all the experiment settings. Oct at concentrations of 0.01 mg/L ,0.1 mg/L,1 mg/L and 10 mg/L,respectively,or 0.01 mg/L Oct + TGF?1,0.1 mg/L Oct+TGF?1,1 mg/L Oct+TGF?1,10 mg/L Oct+TGF?1 were respectively added to the cultured HSCs. Effects of Oct on HSC proliferation and ECM synthesis were respectively determined by MTT method,-TdR and -proline incorporation,or radioimmunoassay. RESULTS: Oct inhibited MTT intake by cultured hepatic stellate cells and down-regulated -TdR incorporation,compared with control group. The concentrations of hyaluronic acid,laminin,collagen type IV in the culture supernatant and -proline incorporation in HSCs were decreased by Oct. TGF?1 obviously up-regulated proliferation and ECM synthesis in cultured HSCs,and Oct significantly blocked these actions. CONCLUSION: Oct inhibited proliferation and ECM synthesis in cultured HSCs,and elicited the effects of anti-hepatofibrogenesis.