Williams syndrome is a rare disease caused by 1.5 ～ 1.8 Mb gene deletion in the chromosome 7q1 1.23 region,with autosomal dominant inheritance.The incidence is about 1/10 000.It involves the cardiovascular system,nervous system,endocrine system and so on,and is most likely to cause damage to the cardiovascular system.The mortality of sudden cardiac death in patients with Williams syndrome is higher than that in healthy population.At present,suspected patients were screened by Lowery score and the American pediatrics score,then were evaluated by imaging examination and were diagnosed by gene detection.The main therapy is symptomatic treatment.Therefore,it is important to ensure an accurate diagnosis and prompt treatment.In the article,the research progress of the clinical diagnosis,gene diagnosis,gene therapy and prognosis is reviewed.

Objective:To discuss the relationship between serum advanced oxidation protein products (AOPP)and cystatin C with the early preeclampsia pregnancy outcome.Methods:Clinical data of 75 patients received treatment and labor at our hospital from 2011 to 2013 was retrospectively analyzed.The control group included 60 normal pregnant women who received prenatal examination in our hospital at the same time.The difference in cystatin C and AOPP level of the patients with preeclampsia and the normal group was compared.The clinical data and the adverse pregnancy outcome of the patients with preeclampsia in different severity and different outcome of pregnancy were compared.Results:A total of 135 patients were retrospective analyzed ,including 75 in observe group and 60 in control group.The SBP level in the patients with preeclampsia is obviously higher than in the normal group .The level of SB,UA,Cr and AOPP in the patients of mild group is obviously lower than in the severe group.The incidence of oligohydramnios , placental abruption,fetal growth restriction and HELLP syndrome in the patients of mild group was obviously lower than in the severe group .The differences were statistically significant.The level of cystatin C,UA,Cr and AOPP in the patients of adverse pregnancy outcome were obviously higher than in the group of good outcome of pregnancy.Conclusion:The cystatin C and AOPP level of the early preeclampsia patients is obviously increased and it has significant correlation with the pregnancy outcome in patients .

Objective To investigate the correlation of Chlamydia trachomatis (Ct) infection with tubal pregnancy by Meta-analysis. Methods Eligible literatures were searched from Chinese websites cnki,wanfangdata and eqvip from 1998 to 2007. Meta-analysis was conducted for the correlation of Ct infection with tubal pregnancy by software RevMan 4.2, and the results were expressed as odds ratio (OR) and 95% confidence intervals (95% CI). Results Thirty-five articles were retrieved, of which 16 were included in the Meta-analysis. The combined ORs of Ct infections in cervix uteri and in uterine tube to tubal pregnancy were7.14 (95% CI: 5.20-9.80) and6.50 (95% CI: 4.52-9.34), respectively. Conclusions There are significant correlation between Chlamydia trachomatis infection and tubal pregnancy.

Objective To compare the consumption of the drugs for patient-controlled intravenous analgesia (PCIA) in patients with different blood groups. Methods One hundred female ASA Ⅰ or Ⅱ patients, aged 30-50 yr, body mass index ＜ 30 kg/m2 , scheduled for elective total hysterectomy or myomectomy, were enrolled in this study. The patients were divided into 4 groups according to the blood group: group A ( n= 25), group B ( n = 30), group AB ( n = 11 ) and group O ( n = 34). Patients received iv injection of flurbiprofen 30 min before the end of surgery and sufentanil at the end of surgery. PCIA with sufentanil 1.2 μg/ml ( bolus dose 2.0 ml, background infusion 0.5 ml/h, lockout interval 5 min) was performed when the VAS score ≤4. The loading dose and consumption on 1 st and 2nd day after operation for sufentanil were recorded. The adverse reactions were observed.Results The loading dose and consumption on 1st day after operation for sufentanil were the highest in group A and B, the second in group AB, and the lowest in group O among the 4 groups. Conclusion The consumption of the drugs for PCIA is different in patients with different blood groups.

In order to get more nutrition from outside the erythrocyte,new channels were induced by malaria par-asite.These channels play an important role in physiology of the parasitized cell.They are of interest both as potential targets in their own right and as potential drug targeting routes capable of mediating the entry of cytotoxic drugs into the app-ropriate compartment of the infected cell.It is hoped that this new anti-malarial strategy will help to create a sustainable anti-malaria-drug-development portfolio for the treatment of malaria.

Objective To explore the influencing factors of job stress of clinical nursing teachers in order to provide information for nurse managers formulating targeted stress management.Method Toally 185 clinical nursing teachers were evaluated with the Chinese nurses stressors scale (CNSS).Results The scores by CNSS was 75.4 ±23.6.Age,working experience,job rank,education background,working department,marriage status and demission intention were found to be adverse impacts on CNSS's scores (all P＜0.01).Conclusion Clinical nursing teachers have middle working pressure.Effective intervention should be provided by nursing managers to prevent and relieve job stress and negative psychological conditions of these teachers.

The paper selects 4 famous foreign Evidence-based Medicine (EBM) databases-UpToDate,DynaMed,Medskills and CISMeF for analysis and comparison from 3 perspectives of the structures,retrieval methods and evidence grading standards,in order to provide reference for the construction and development of the EBM databases in China.

Pulmonary artery hypertension is a serious disease in respiratory system and a key tache in the mechanism of pulmonary-heart disease. The pathological changes include the contraction and remodeling of the pulmonary vessels. There are more and more studies on the pulmonary artery hypertension because of its refractory character and the following increasing mortality. This article summarizes the updating research of the factors and mechanism studied on pulmonary artery hypertension recently, to provide a new view for the clinical and basic medical investigation.

The comprehensive analysis and comparison of the application and the technical character of a variety of hematology analyzer widely used in China's medical institutions is proposed on the basis of an introduction to the principle and technical features involved in the five-classification technology of the hematology analyzer, and the combined analysis on behalf of the five-classification technology is focused in above. Finally, the development trend of the hematology analyzer is summarized.

Objective To determine the potential deficiency due to lack of anaerobic culture and evaluate the effect to reduce adverse reaction associated to transfusion-translated bacterial infection.Methods The result of 9 758 units of apheresis platelet concentrates (PCs)detected with automated microbial detection system were reviewed and the medical records of the patients that received the contaminated PCs were followed.Results The confirmed positive rates by aerobic and anaerobic cultures were 0.06％ (6/9 758)and 0.16％ (16/9 758),respectively.In 10 of 16 yield cases,only the anaerobic culture was positive.The most of the bacterial detected by anaerobic culture only were Propionibacterium acnes.Their mean detection time from inoculation was 96.8±18.21 hours.Conclusion Addition of anaerobic culture would enhance the detection of bacterial contamination in PCs.However,since only slow-growing bacteria were detected,and because their clinical significance was debatable,blood service should select feasible and costeffective projects using only aerobic bottle for bacterial screening,like the majority of licensed blood centers in North America and Hong Kong,China.