Objective To study the gene mutation and clinical characteristics of hereditary spinocerebellar ataxia type 7 (SCA7).Methods The regions of SCA7 gene containing CAG repeat were amplified by means of polymerase chain reaction (PCR) and polyacrylamide gelelectrophoresis (PAGE) technique in 112 patients with autosomal dominant SCA from 92 families, 16 sporadic SCA patients, 71 family members and 60 healthy controls. The abnormal allele fragments were sequenced by ABI377 DNA sequencing machine. The correlation between clinical manifestations and CAG repeat size in SCA7 gene product was analyzed. Results 2 patients of 6 members in 1 SCA7 family carried a 71-repeat allele. The main clinic features included ataxia, hypopsia, axanthocyanopsia and retinal pigmental degeneration. Alleles from 7 to 9 repeats were seen in the other 4 healthy members. CAG repeats from 6 to 21 were found in other 126 SCA patients, 71 family members and 60 healthy controls. Conclusions Expanded triplet repeats in SCA7 gene contributes to the pathologic phenotype, and molecular genetic analysis is effective for the diagnosis of SCA7. Retinal pigmental degeneration is an important characteristic of SCA7.

Objective To analyze the value of pulse sequential continuous cardiac output(PICCO) in chronic pulmonary heart disease with cardiogenic shock.Methods Sixty-eight patients with chronic pulmonary heart disease complicated with cardiogenic shock were randomly divided into study group(n=36) and control group(n=32).Both groups were treated with conventional therapy,while the control group underwent deep venous puncture to monitor the central venous pressure (CVP) changes.The study group underwent deep venous catheterization+ femoral artery catheterization monitoring + PICCO monitoring.The therapeutic effect and the changes of PICCO index of the two groups were observed.Results The time of administration of vasoactive drugs,hospital stay and mechanical ventilation were significantly lower in the study group than in the control group (P＜0.05).There was no significant difference in mortality rate between the two groups(P＞0.05).After treatment,cardiac output quantity (CI) in study group increased significantly and extravascular lung water index EVLWI and PVPI decreased significantly,the difference between the two groups were statistical significance (P＜0.05),ITBVI had no statistical significant difference betweent two groups(P＞0.05).CI in the survival group was significantly higher than the death group,PVPI and EVLWI were significantly lower than the death group (P＜0.05),and the two groups had no significant difference in ITBVI (P＞0.05).Conclusion PICCO can reflect the hemodynamic status of patients with chronic pulmonary heart disease complicated with cardiac shock,and it has important clinical value for guiding the treatment and prognosis of patients.

ObjectiveTo evaluate the efficacy of axillo-axillary bypass grafting for the treatment of contralateral arterial ischemia of upper limb. Method 53 cases underwent axillo-axillary artery PTFE graft bypass among those there were 46 cases of subclavian artery occlusion and 3 cases of subclavian artery aneurysm and 4 cases of trauma. Preoperatively 33 cases suffered from a symptom of extremity ischemia and 18 cases with subclavian artery blood steal. Result Good results were achieved in all cases without any complications. Doppler test showed the gradient of bilateral brachial pressure artery was less 10 mmHg in all cases. ConclusionAxillo-axillary artery PTFE graft bypass is an effective method for subclavian artery reconstruction with less trauma and complications. It is especially suitable for the elders with concomitant heart brain and lung diseases. It is also applicable for cases in which the subclavian arteries are not easily accessible due to artery aneurysm and artery trauma.

Objective To develop coronary artery disease self-management scale and evaluate its reliability and validity.Methods The item pool was based on the theory of chronic disease self-management and the evidence of behavioral medical science related to coronary heart disease.Firstly,the preliminary scale was developed through specialist evaluation;then it was reviewed by 10 patients and specialists again;At last,209 patients were surveyed and based on the pilot data,a series of statistic analysis was conducted such as factor analysis,correlation analysis,etc.To deduce the items to 27 and then a comprehensive set of psychometric tests was performed on the final version.Results Psychometric tests with the application of stringent criteria confirmed the test-retest reliability(ICC=0.910),internal consistency(Cronbach's ?=0.913),criterion-related validity(r=0.271-0.573),construct validity(factor analysis confirms the hypothesis of the scale),responsiveness(t=9.011,P

Objective To investigate the effect of various adjunctive drugs in patients undergoing abdominal surgery under epidural anesthesia. Methods 60 ASA Ⅱ～Ⅲ patients scheduled for elective abdominal surgery were randomly divided into four groups: Group PP, promethazine 50mg + ephedrine 100mg as one unit; Group DF, droperidol 5mg + fantanyl 0.1mg as one unit; Group MP, midazolam 5mg + pethidine 100mg as one unit; Group MF, midazolam 5mg + fantanyl 0.1mg as one unit. All the drugs were intravenously administered 10 min after the first dose of epidural block. The dose of various combinations was selected to reach sedation degree of Ramsay 4. The blood pressure, heart rate and SpO_2 were monitored, the degree of sedation and the side-effects were recorded during the operation. The patients were postoperatively surveyed to evaluate the degree of amnesia and satisfaction of anesthesia. Results No significant differences in hears rate and SpO_2 were found among the four groups. In group DF there was marked influence on the blood pressure. The time needed to reaching Ramsay 4 degree of sedation in MP group (1.49?1.13min) and MF group (1.64?0.96min) was significantly shorter than that in group PP (4.36?2.11min) and group DF (4.97?2.65min) (P≤0.01). An obvious difference was found in the incidence of responses to surgical exploration. There was a higher risk of glossoptosis in groups MP and MF than in other groups. Conclusion Midazolam combined with fentanyl or ephedrine is the most effective combination to supplement epidural anesthesia for abdominal operations.

Objective To evaluate the prediction value of the early prediction score system of multiple organ dysfunction syndrome in the elderly (MODSE). Method A total of 393 patients with pulmonary infection, who were above60 years old, were divided into non MODSE(n =224) and MODSE group(n = 169) and were scored by the early prediction score system of MODSE. Independent-samples t Test was used to analyze the difference of forecast score between MODSE and non MODSE group. Receiver operating characteristic (ROC) curve was drawn,and the area under the curve was calculated. The prediction accuracy of scores for MODSE was assessed using sen-sitivity and specificity, and the optimal forecast point for MODSE was found. Results The score of MODSE group was higber than that of non MODSE[(19.38±12.049) vs. (45.78±20.257), P <0.001]. The area under the ROC curve was 0.889 (P <0.001) ond 95% (0.857～0.920). As the value of forecast score was 27.5, the sensitivity of the early prediction score system of MODSE was 82.8 %, the specificity of the early prediction score system of MODSE was 80.3 %. Conclusions The early prediction score system of MODSE is valuable in predic-tion of MODSE, which may be used to forecast MODSE and find the high risk population of MODSE.

Objective To evaluate the therapeutic effect of sho-saiko-to silymarin on liver fibrosis due to chronic hepatitis B.Methods 90 patients with liver fibrosis due to chronic hepatitis B virus(HBV) were randomly divided into therapy group and control group.50 patients in therapy group were given sho-saoko-to and silymarin;While 40 patients in control group were given silymarin only,they were all treated for 6 months.The changes were observed including liver function,serum liver fibrosis and virology indexes before and after treatment in two groups.Resuits Indexes of liver function and serum liver fibrosis in therapy group were improved obviously(P<0.05 or P<0.01),while better clearance to HBV is comparable to control group(P<0.05).Conclusion The treatment with sho-saiko-to combined silymarin on liver fibrosis due to chronic hepatitis B had explicit clinical effect.

Objective Objective To evaluate the efficacy and comphcations of late course acceler- ated hypofractionated three dimensional conformal radiation therapy (3DCRT) for patients with stageⅢnon small cell lung cancer (NSCLC). Methods Sixty patients with stageⅢNSCLC were randomized into 2 groups: Late course accelerated hypofractionated 3DCRT group(group A—30 patients) and conventional fractionated radiation therapy group (group B—30 patients). In group A, 30 patients, at first, received a dose of 40 Gy at 2 Gy per fraction, 5 times a week, which followed by late course accelerated hypofractionat- ed 3DCRT with a dose of 16-20 Gy at 4 Gy per fraction, 3 times a week. In group B, 30 patients received a dose of 60-66 Gy at 2 Gy per fraction, 5 times a week. Chemotherapy, including vinorelbine and cisplatin, was given one cycle during radiotherapy and 3 cycles after radiotherapy for all patients. Results Group A had a higher complete response rate (47% vs 20%, P

Aim To observe the effect of kanamycin on the change of Ca2+ concentration in outer hair cells caused by acetylcholine to explore the relationship between kanamycin ototoxicity and the cochlear efferent nervous system.Methods After the guinea pigs were killed by decapitation,the OHCs were isolated in artificial perilymph and divided into three groups:control group,acetylcholine(ACh) group and kanamycin + ACh group.OHCs were loaded with Fluo-3/AM for 30 min and then the change of Ca2+ fluorescence intensity was observed.Results Ca2+ fluorescent intensity in OHCs remained basically unchanged in control group,increased 3.06 times(n=6) in ACh Group,and increased 1.75 times(n=6) in kanamycin +ACh Group.Conclusions Kanamycin can obviously inhibit the increasing of Ca2+ concentration in OHCs caused by ACh.

Objective To investigate the clinical value of acoustic radiation force impulse (ARFI) imaging in the evaluation of liver functional reserve in patients with hepatic focal lesions. Methods Eighty-six patients with hepatic focal lesions in General Hospital of the People′s Liberation Army from September 2011 to August 2012 were studied. According to Child-Pugh score, 54 of them were classified as grade A, 18 patients were classiifed as grade B, and 14 patients were classiifed as grade C. Seventy patients underwent surgery and 16 patients underwent non-surgical treatment. Shear wave velocity (SWV) values were measured using ARFI technique, and indocyanine green (ICG) clearance tests were also performed to achieve ICG clearance rate constant (ICGK) and ICG retention rate at 15 minutes (ICG15). Correlation between SWV and ICGK, SWV and ICG15 were studied using Pearson correlation, and correlation between SWV and Child-Pugh score were analyzed using Spearman rank correlation. Difference between SWV values of patients underwent surgery and those of patients underwent non-surgical treatment was compared using t-test for independent samples. Analyses of receiver operating characteristic (ROC) curve were performed to investigate the value of ARFI in the evaluation of surgical feasibility for patients with hepatic focal lesion, with the clinical decisions serving as the golden standard. Results There was correlations between SWV and ICGR15, SWV and ICGK, and SWV and Child-Pugh scores (r=0.764,P <0.001;r=-0.686,P=0.000;r=0.864,P=0.000). The SWV of patients who underwent surgery was (2.46±0.45) m/s, and the SWV of patients who underwent non-surgical treatment was (1.54±0.36) m/s, with a signiifcant difference (t=-0.80, P=0.000). The ROC curve analysis demonstrated a highest Youden′s index of 0.775 when the cut-off was 2.06m/s. The corresponding sensitivity was 87.5% and the specificity was 90.0% for ARFI in the diagnosis liver functional reserve deficiency. Conclusion ARFI can effectively evaluate the liver functional reserve in patients with hepatic focal lesions and thus could be beneifcial in the decision of clinical treatment.