Objective To develop a scale to assess Kennedy's disease (KD) based on patients' clinical characteristics and to validate its application in patients.Methods We developed KD1234 Scale following the model of Amyotrophic Lateral Sclerosis-Functional Rating Scale.Using this new scale,we assessed patients with genetic diagnosis of KD and evaluated the reliability and validity of the new scale.Reliability was analyzed using Cronbach' s α coefficient and split-half reliability.Validity was analyzed by content validity and structure validity.Results The Cronbach' s α coefficient of the total scale was 0.789 and split-half reliability coefficient was 0.868.The α of breathing with total score was 0.3-0.4,α of written,language and swallowing with the total score was 0.4-0.5,α of other items with the total score was ＞ 0.5 (P ＜ 0.01 in each of above α).Four common factors extracted by exploratory factor analysis had well correspondence between the scale construction and the theoretical construction.Factor loading was ranged from 0.541 to 0.864 for each item.The duration of the disease showed some correlation with the score of KD1234 Scale.Conclusions The KD1234 Scale demonstrates high reliability and validity.The duration of the disease indicates negative correlation with KD1234 Scale scores.The KD1234 Scale can be used in clinical research to evaluate the condition of KD patients quantitatively.

Objective To explore the significance of serum myoglobin detection in the diagnosis and evaluation of Kennedy`s disease (KD).Methods The level of serum myoglobin (Myo) was detected in 60 KD patients and 30 amyotrophic lateral sclerosis (ALS) patients.Results The serum Myo level and abnormal rate in KD group were significantly higher than those in ALS group (all P<0.001).The serum Myo level was positively related with the disease course of KD (r=0.665,P<0.01).There was no significant difference of serum Myo level and disease course of ALS (r=-0.047,P>0.05).There was no overlap of serum Myo level between the two groups.There was no significant difference of serum Myo level and CAG repeated number of ALS (r=-0.193,P>0.05).Conclusion Myo is likely as an easy and sensitive biomarker, used to identify the KD and special type of ALS, and used in the evaluation of the KD condition in the future.

Objective To study the chemical constituents ofPronephrium triphyllum.Methods The chemical constituents in the plant were isolated and purified with silica gel and Sephadex LH-20.Their structures were identified by analyses of spectral data and physicochemical properties.Results Six compounds were isolated and identified as shelincaoide A(1),n-butyl-13-D-fructopyranoside(2),triphyllin A(3),6,7-di-hydroxycoumarin(4),daucosterol(5),and 13-sitosterol(6),respectively.Conclusion Compound 1 is found to be a new compound.Compounds 2 and 4 are firstly isolated from the plants in Pronephrium Presl.and all compotmds except 3 are obtained from the species for the first time.

Objective To investigate the role of miR-10a in CD4+CD25+Treg-mediated immunosuppression during sepsis and its potential role in immunotherapy for sepsis.Methods Sepsis mouse model was established by cecal ligation and puncture(CLP).Balb/c mice of clean grade were sacrificed 1,3,5,and 7 days after operation.Blood as well as spleen samples were harvested at given intervals.The splenic CD4+CD25+Treg cells and CD4+T cells were isolated by MACS microbeads.Cells were cultured,and phenotypes were analyzed by flow cytometry.The miR-10a expressed in Treg cells were detected by Real-time PCR.After administration of LV-mmu-miR-10a-5p-inhibition,the immunosuppressive function have been detected.Statistical analyses were performed using one-way analysis of variance (SPSS 19.0,Chicago,USA) test followed by Dunnett-t test to compare among three or more groups or by Student's t-test to compare between two groups.Results The percentages of splenic Tregs (CD4+CD25+/CD4+T) was (7.34±1.2)％ in normal group,and the increase in percentage of Tregs in spleen has been observed in septic mice (P＜0.05).The mean fluorescence intensity (MFI) of Foxp3+Treg was increased in septic mice compared with sham group (P＜0.05).The expression of miR-10a was significantly elevated on CLP 1-7 day (P＜0.05).After down-regulation of miR-10a in septic mice,the percentages of Tregs (CD4+CD25+/CD4+T) was significantly increased in septic mice (P＜0.05),the MFI of Foxp3+Treg was increased in septic mice compared with control group (P＜0.05).The CD4+T cell proliferative activity in CLP-induced mice was significantly suppressed on CLP 3 day compared with sham group (P＜0.05).After down-regulation of miR-10a in septic mice,the CD4+T cell proliferative activity was significantly suppressed compared with control group (P＜0.05).Conclusions Treg plays a critical role in immunosuppression in septic mice.Inhibition of miR-10a in vivo could enhence immunesuppression of CD4+CD25+Treg.Therefore miR-10a may participate in the regulation of CD4+CD25+Treg immunosuppression in sepsis and become the target for immunotherapy.