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 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.

Hypoxia-induciole factor-1 (HIF-1) is an important transcription factor.It participates in the body's response to the hypoxic environment mainly through its active subunit HIF-1α.During cerebral ischemia and hypoxia,the expression of HIF-1α is upregulated and may activate the expressions of multiple downstream target genes involving glycolysis,vascular growth,cell survival and apoptosis.It has an important significance for the improvement of energy metabolism and the establishment of microcirculation after cerebral ischemia.HIF-1α can not only promote the neuronal survival,but also mediate delayed neuronal death.A variety of preconditioning and postconditioning methods may regulate the survival and death of neuronal cells by activating HIF-1α.

The imported schistosomiasis cases in the Chinese literature and reports from the infectious disease monitoring in-formation system from 1979 to 2013 were collected and compiled. Totally 365 cases were reported to be infected with outside schis-tosomiasis, including 239 cases of schistosomiasis haematobia (74.0%) and 71 cases of schistosomiasis mansoni (22.0%), and 42 foreigners (11.5%) and 323 Chinese (88.5%). The infection areas involved 15 countries and regions in Africa. Totally 74.92%of pa-tients worked in the construction or geological prospecting in the wild field. According to the analysis of these cases, we found that the management system of prevention and control of imported schistosomiasis cases was not perfect including the high missing re-port rate, high misdiagnosis rate, and no standard diagnostic criteria. We suggest that all the levels of CDC or health administrative authorities should adopt the following relevant control measures to strengthen the imported schistosomiasis prevention and control in order to reduce the damage to the public health and the risk of the spread of African schistosomiasis in China:1. to establish and perfect the imported schistosomiasis monitoring and control system in China;2. to integrate the dynamic information platform of la-bor export and establish the comprehensive prevention and control management system of infectious diseases;3. to standardize the diagnosis and treatment of oversea imported schistosomiasis; 4. to strengthen the research on the transmission risk of imported schistosomiasis in the territory of China.

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.

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.

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.

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.