Objective To investigate the clinical and electrophysiology features of infantile spinal muscular atrophy,and explore the clinical significance of genetic diagnosis. Methods The clinical data of 13 infants suffering from infantile spinal muscular atrophy were analysed.The serum creatine phosphokinase was examined,and nerve conduction velocity was tested in median nerve,ulnar nerve,tibial nerve and peroneal nerve.The parameters such as distal motor latency,motor nerve conduction velocity and amplitude of compound motor active potential were analysed.Electromyography was performed in no less than four muscles,and the insertion potential,spontaneous potential and motor unit action potential were observed.Deletion of exon 7 in SMN1 gene was detected by polymerase chain reaction-restriction fragment length polymorphism(PCR-RFLP). Results All these infants were characterized by progressive flaccid paralysis in limbs.In all cases,amplitude of muscle response was significantly decreased,with prolonged distal latency and slowed conduction velocity.Electromyography demonstrated motoneuron degeneration.Deletion of exon 7 in SMN1 gene was detected in all 13 infants. Conclusion There are unique clinical and electrophysiology features for infantile spinal muscular atrophy,and electromyography may play an important role in the diagnosis.Prenatal genetic diagnosis may help to avoid the birth of this kind of infants.

Objective: To study the diagnostic characteristics of cardiac amyloidosis (CA) by non-invasive electrocardiography (ECG) in relevant patients. Methods: We retrospectively analyzed 60 CA patients diagnosed in our hospital from 2008-08 to 2013-12 for their clinical and ECG characteristics. Results: There were 48 male and 12 female patients with the ratio of 4: 1. The ifrst time diagnosis rate was low and the average age for conifrmed diagnosis was at (54. 5±14. 2) years.①There were 32 (53. 3%) cases combining heart failure, 12 (20%) with pleural effusion, 20 (33. 3%) with atrial arrhythmia, 8 (13. 3%)with ventricular arrhythmia, 4 (6. 7%)with sino-atrial block, 15 (25%)with atrio-ventricular block, 4 (6. 7%) with left bundle branch block (LBBB), 5 (8. 3%)with RBBB and 8 (13. 3%)with intra-ventricular block.②There were 32 (53. 3%) cases with low voltage on limb leads, 52 (86. 7%) with pseudo-infarct pattern, 48 (60%) with ST-T abnormality and 30 (50%) combining low voltage on limb leads with pseudo-infarct pattern.③The patients combining pleural effusion and with pseudo-infarct pattern had the increased ratio of low voltage on limb leads, while there were still 22 (45. 8%) cases without pleural effusion had low voltage on limb leads.④ ECG characteristics for 60 CA patients were as follows: QRS duration (104±26) ms, QT interval (404±34) ms, QTc (462±35) ms; the R wave of avR 0. 17 mV, QRS wave 0.30 mV; the R wave of limb leads and V1-3 were all<0.5mV, the S wave of V1-3 were 0. 62mV, 1. 61mV, 1. 56mV; the R/S ratio of V1-3 were 0. 19, 0. 12, 0. 20 respectively. Conclusion: CA patients had the highest incidence of pseudo-infarct pattern; meanwhile, combining with low voltage on limb leads, pseudo-infarct with long Q or S wave and ST-T abnormality but normal QRS duration was helpful for differential diagnosis of CA in clinical practice.

Objective To investigate the effect of plasma homocysteine (Hcy) levels and cognitive dysfunction on patients with type 2 diabetes and explore the other factors that affect cognitive function.Methods 80 patients with type 2 diabetes were selected as our subjects and they were divided into cognitive impairment group (38 cases),cognitive normal group (42 cases) according to a simple mental state scale(MMSE) score.The plasma Hcy,glycosylated hemoglobin,blood lipid,uric acid,24 h urine trace albumin were measured.Results Plasma Hcy concentration in diabetic cognitive dysfunction,non cognitive impairment group and normal control group respectively were(19.56 ± 5.23),(16.21 ± 3.27),(14.67 ± 4.27) tmol/L,and there was statistically significant difference(F =3.76,P ＜0.05).The plasma Hcy levels in diabetic cognitive impairment group was higher than that non-cognitive impairment group(P ＜ 0.05) and normal control group (P ＜ 0.01),The factors impaired cognitive function in patients with other factors included age,glycosylated hemoglobin,body mass index,diabetes duration and plasma Hcy levels,and there were negative correlation with MMSE score (r =-0.336,-0.285,-0.226,-0.392,-0.312 ; all P values were less than 0.05).Conclusion Patient's age,duration of diabetes,glycosylated hemoglobin and BMI can affect cognitive function in patients.Higher Hcy levels in patients with type 2 diabetes is a risk factor for cognitive impairment.

Objective: To know the situation of college students to grasp knowledge about generation health in Xi'an and to help the department to give an aimed education. Methods: 1020 students were investigated by questionnaire and the data be analyzed by the software Epi5.0. Result: College students' knowledge about generation health and STDs were very little. About 1/3 of them didn't know how to make urgent contraception, however the proportion of abortion were so high in university. Conclusion: The education of knowledge about generation health and guidance about contraception should be strengthened in university, especially in freshmen.

Object: To know the situation of college students' knowledge about STDs and to help special department to give an aimed education. Methods: 1020 students were investigated by questionnaire and the data analyzed by the Epi5.0. Result: College students' knowledge about STDs were very little. Just 12.2% of these students know exactly the name of STDs which should be key prevent. About the knowledge of transmission and prevention of STDs, the married, older, higher educated and medical students were better than the corresponding other students. Conclusion: The education of knowledge about STDs should be strengthened in university, especially for freshmen.

Objective: To understand AIDS knowledge of the college students,to provide scientific evidence for making out the strategy of health education and synthetic prevention measure purposely.Methods An anonymous questionnaire was conducted with face to face interview to investigate the college students in Xi'an, and the software Epi info 5.0 was used to perform the statistical analysis of the data. Results 70.5% of university students knew the whole name of AIDS. As to three ways of HIV transmission, the investigation shows that 49.1% of college students knew three main ways. The 26.5% of college students mistakenly think that mosquito could transmit HIV. In general, the old, the married the literate, and medical students get more knowledge about AIDS than the young,the unmarried,the illiterate, and other major students. Conclusion The college students who are one of the most dangerous groups infected AIDS have poor knowledge. The situation of poor AIDS knowledge is extremely unfit to the prevalent tendency of AIDS, which our country is being faced. We suggest that the college students should be educated intensively on the health knowledge of AIDS especiallis beingy for freshmen.

Objective To explore the effect of 14,15-epoxyeicosatrienoic acids (14,15-EET) on the inflammatory response of BV2 cells under oxygen and glucose depriviation/reoxygenation (OGD/R) conditions.Methods BV2 cells were randomly divided into three groups,blank control group,vehicle control group,and 14,15-EET group.Under treatment of 14,15-EET,the concentration of inflammatory factor in BV2 cell culture media was detected by ELISA at different time points (reoxygenation for 0,3,6,12,24 h) after OGD1h.The viability of BV2 cells was detected by MTT assay at different time points.At the same conditions,using Transwell migration experiment,migration ability of BV2 cells was observed.Results The 14,15-EET group had the lower levels of inflammatory factor secretion,lower viability and weaker ability of migration than the vehicle control group.The above results were most statistically significant at OGD1h/R12h.Conclusion 14,15-EET can inhibit the inflammation of BV2 cells induced by the injury of OGD reperfusion.

Objectives To discuss the characteristics of late gadolinium enhancement (LGE) magnetic resonance (MR) imaging in patients with hypertrophic cardiomyopathy (HCM) and left ventricular apical aneurysm (LVAA) and its related prognostic value. Methods Thirty HCM patients with LVAA were collected from August 2004 to August 2013. All cases with coronary artery diseases were ruled out, and all patients underwent LGE derived by cardiac MR (CMR). Five cases of LVAA were pathological confirmed. Atrial and ventricular diameters, apical aneurysm diameters and left ventricular ejection fraction were measured, and apical aneurysm LGE was evaluated. All patients were then followed up. Comparisons in continuous parameters between patients with or without LGE were performed by independent t test. A Cox proportional hazard model was used to estimate the hazard rate for adverse cardiovascular events. Results LGE was identified in 21 LVAAs and non-LGE in 9 LVAAs. Between two groups, there was no significant difference in the size of LVAA [(16.4 ± 11.5) mm vs. (20.3 ± 9.8) mm, P=0.63]. In particular, the complete transition from non-LGE to LGE LVAA was recorded during follow-up in one patient. Pathological findings confirmed that LGE indicated fibrous tissue in LVAA, and LVAA without LGE indicated viable myocardium. The follow-up suggested that the patterns and the size of LVAA were associated with the adverse events in these patients (LGE of LVAA, HR=7.94, P=0.064; the size of LVAA, HR=1.08, P=0.009). Conclusions LGE-MR had important clinical significance in HCM patients with LVAA. LGE in LVAA corresponded with the fibrous tissue and was associated with the prognosis.

Objective To clarify the MRI characteristics and outcomes of patients with end stage hypertrophic cardiomyopathy (ES-HCM).Methods Clinical and MRI data of 57 ES-HCM patients were retrospectively analyzed.ES-HCM pa tients were divided into dilated phenotype group (D-ES group,n=39) and restrictive phenotype group (R-ES group,n=18).MRI characteristics and outcomes of patients were compared between both groups.Results The incidence of atrial fi brillation and edema of lower extremity was significantly higher in R-ES than those in D-ES (72.22％ [13/18] vs 30.77％ [12/39];50.00％ [9/18] vs 23.08％ [9/39];both P＜0.05).The left ventricular ejection function,left and right atrial anteroposte rior diameter of D-ES group were significant smaller than those of RRES group (all P＜0.05),while the left ventricular (LV) short axis diameter,LV end diastolic/systolic volume and LV end diastolic/systolic volume index of D-ES were significantly greater than those of R-ES group (all P＜0.05).Log-rank test found no significant difference between both groups in cardiovascular death/ heart transplant events (x2 =1.135,P=0.287).Late gadolinium enhancement (LGE) volume fraction was significantly larger in D-ES ([36.1±14.8]％) than in R-ES ([21.0±9.0]％;P＜0.05).There was a significant correlation between LGE volume fraction and cardiovascular death/heart transplant events (HR:1.054,P＜0.05).Conclusion ES-HCM patients have expanded clinical expression and MRI characteristics,including dilated phenotype and restrictive phenotype.MRI has an important application value in the diagnosis and prognosis evaluation of ES-HCM.

ObjectiveTo investigate the effects of autologous bone marrow-derived mesenchymal stem cells (MSCs)transplantation on acute myocardial infarction in swine models using MRI. MethodsFourteen Chinese mini-pigs(27±3 kg)were divided into control group(n=7)and transplantation group(n=7).Acute myocardial infarction(AMI)model was made by occlusion of the left anterior descending coronary artery for 90 minutes,and then 10 ml autologous MSCs(3 × 106 cell/ml)were injected into LAD by over-wire-balloon catheter after one week. MRl was performed to assess the cardiac function and myocardial perfusion 1 week after AMI and 6 weeks after transplantation.The implanted cells in vitro were analyzed by immunofluorescence.ResuitsThe left ventricular ejection fraction(LVEF)in transplantation group was increased from(42.7 ±7.5)%to(50.1±10.1)%,which was significantly different from that in control group(P＜0.01).In addition,the dyskinetic segments in infarcted region and the infareted area were decreased by 4 and 3.2 cm2 respectively(P＜0.01),and the left ventricular weight index was increased by 4.1 g/m2 in transplantation group(P＜0.05)compared with control group.The DAPI-labeled cells in infarcted and peri-infarcted region indicated the survived MSCs.Immunofluoreseence also confirmed that those cells expressed cardiomyocyte-specific troponin T,connexin 43 and vessel-specific smooth muscle actin.Capillary density in both infarcted and peri-infarcted region were higher in transplantation group than the control group(P＜0.01).Conclusion MRI is a reliable imaging method for assessing the effects of stem cell transplantation in acute myocardial infartion of swine models.