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 evaluate MRI characteristics of soft tissue hemangioma.Methods 30 cases of soft tissue hemangiomaconfirmed by surgery,angiography and clinical diagnosis included in this study.All cases underwent plain MRI scans,24 cases of them were further-examined by contrast-enhanced MR scans.Results 24 cases were cavernous hemangioma,4 cases were capillary hemangioma and 2 were tufted hemangioma.The tumors showed moderate signal on T_1WI in 24 cases,and significantly higher signal on T_2WI in all cases(even stronger than that of fats).The vascular component within the tumor displayed remarkable enhancement and appeared as serpentinevessels,while the non-vascular tissues showed no enhancement after intravenous administration of contrast agent.Conclusion MRIexamination is of great importance in determining the position,the nature and the extent of hemangioma as well as the effectiveness ofoperation.

The Hospital of Xinle Sector of Xuhui District in Shanghai started implementing the 2000 version of ISO9001 from September 2000. In doing so it highlighted respectively the following aspects: preparations for the implementation, intensified training, compilation of documents on the quality system, operation of the quality management system, examination and verification of the internal quality system, and examination and verification of attestation. As a result of conscientious implementation, the hospital was issued a certificate for its quality management system in May 2001 by the Quality Attestation Center of the Square & Round Mark Attestation Commission. The hospitals chief experience is that implementation of ISO9001 is a new means of constructing a model of modern medical quality management; it is a prerequisite for the sustained development of a hospital; and it guarantees organizationally the effective operation of medical quality management.

Recent discoveries about the effects of drugs of abuse on the brain and the mechanisms of their addictions, and new actions of available medications are offering many opportunities for the discovery and development of novel medications to treat addictive disorders.This article reviews the current medications that have shown promising results for treating opioid, cocaine, methamphetamine, cannabis, alcohol and nicotine addictions.

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.

Objective To study the current situation of diagnosis and treatment of rudimentary uterine horn pregnancy through 4-cases report. Methods We made a clinical study of 4 cases of rudimentary uterine horn pregnancy admitted to our hospital between 1995 and 2005. Results Three patients had been undergone artificial abortion before they were admitted to our hospital. All the patients were asymptomatic. Ultrasonography was made in 3 cases. Two cases were diagnosed as rudimentary uterine horn pregnancy. Three patients were cured with surgery. Conclusions Rudimentary uterine horn pregnancy is rare and with atypical clinical manifestation in the first trimester. Although diagnosis before surgery is difficult, ultrasonography and MRI make a great role. Surgery is the most important therapeutic method.

Objective:To test Jiang Guangrong's(2005)three-dimension model on the depth of counseling process.Methods:In this process-outcome research,28 sessions in a university counseling center were videotaped and coded in terms of the model.Structure Equation Model(SEM)was used to examine the relations between the coding and counselors and clients' evaluation of the session depth.Results:(1)Clients and counselors evaluated differently on the session depth,and clients' evaluation was more positive than counselors' [(4.95±1.12)vs.(4.23±0.94),P<0.05];(2)The model was significantly correlated with counselors' session depth evaluation(path coefficient = 0.55,P<0.01),but not with the clients';(3)The session curves describing each dimension changing over time had different patterns in sessions of different depth level,through which more about the counseling process could be known.Conclusion:The three-dimension model on the depth of counseling process has got some empirical evidences.

BACKGROUND: Animal models of peripheral partial epiphyseal plate closure are commonly used in related trials.OBJECTIVE: To modify the original animal models of distal femoral partial epiphyseal plate closure.DESIGN, TIME AND SETTING: A self-controlled trial was performed at the Experimental Animal Center of General Hospital of Chinese PLA from March to October 2007.MATERIALS: Twenty healthy New Zealand rabbits of 4-6 weeks old weighing 1.0-1.5 kg were selected.METHODS: A straight incision about 2 cm was made in lateral knee of 20 rabbits. The subcutaneous tissues and knee capsule were cut open to expose distal femoral condyle. The condylar plate line was clear. Two holes were drilled in the fight lateral condyle, about 3.0-4.0 mm deep, and partial normal epiphyseal plate (about 33%-44% of the total growth plate) was removed. An incision deep to periosteum was created in the left side and regarded as self-control.MAIN OUTCOME MEASURES: Femoral length, valgus angle of the distal femur, and femoral deformity were detected by X-ray photograph, and gross observation. Bone bridge formation of bilateral femora was observed by histological examination.RESULTS: One rabbit died of diarrhea 31 days after surgery, and 19 were included in final analysis. ①Radiographs showed that the mean length of left femur (control side) was significantly longer but valgus angle was significantly smaller than the right side (experimental side) 4 months after surgery (P ＜ 0.01). The left distal femur averagely grew for 2.27 cm,accounted for 57.6% of the overall femoral growth, and the right side averagely grew for 0.45 cm, accounted for 21.4% of the overall femoral growth.②Four months after surgery, femoral appearance of the control side was normal, with smooth and complete condylar and facies articularis patellae. While, the experimental side femur was significantly shortened, and severe vaigus deformity was observed in the distal femur. ③Five weeks after surgery, layer cells of the epiphyseal plate arranged regularly in the control side. Bone trabecula with many fibers was found in bone defect cavity of the experimental side, and the cell layer of the remnant epiphyseal plate was decreased. The epiphyseal plate was closed in the control side,and bone trabecula of the experimental side was thickened 10 weeks postoperatively.CONCLUSION: This modeling method is simple, efficient, and easy to manipulate; in addition, the bone bridge area is controllable. The models can meet the study demands for peripheral partial epiphyseal plate closure, and are significant for filling material selection during treatment.