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 .

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.

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.

Subject-oriented and specialization information service is a new service model adapting to new information environment and catering to demands of customers. On the basis of the practices and researches of subject-oriented and specialization information service in Guang'anmen hospital, this article put forward that providing the information service has positive significance for accelerating the construction of information resource, improving the level of information service and enhancing the core competitiveness of hospitals.

Objective To discuss the application and effect of sufentanil combined with propofol on painless gastroscopy anesthesia.Methods In the fourth people's hospital of Zhejiang in April 2014-June 2016,94 cases treated with painless gastroscopy I were randomly divided into two equal groups.Anesthesia group A were anaesthetized with fentanyl and propofol,sufentanil and propofol for anesthesia group B.The anesthetic effect,the time of consciousness disappearance,eye opening time,recovery time of orientation,the dosage of propofol in the two groups were compared.The mean arterial pressure,heart rate and adverse reaction rate in the two groups were compared.Results The anesthetic effect in the anesthesia groupB is significant better than that in the anesthesia group A(P<0.05).Compared with the anesthesia groupA,the time of consciousness disappearance,eye opening and orientation recovery is shorter in the anesthesia groupB(P<0.05),The difference of the mean arterial drug Pressure and heart rate is not significant in the two groups before medication.After medication and wake up,compared with the anesthesia group A,the mean arterial pressure,heart rate fluctuation is smaller,and the adverse reaction rate is lower in the anesthesia group B,(P<0.05).Conclusion It is effective which sufentanil combined with propofol used in anesthesia in painless gastroscopy,It can stable vital signs of patients during operation,,work quickly,restore consciousness rapidly,and contribute to reduce complications,and is worthy of promotion.

Total 12 478 of newborn babies were included in this study during the period of from 2001 to 2008 in Yanbian Maternity and Infant Health Hospital.The incidence of natal teeth was 0.96%,and it was 1.65% and 0.31% in Korean nationality and Han nationality,respectively.The difference of natal teeth incidence between Korean nationality and Han nationality was statistically significant.

39 cases of fatally abused children were collected during April,1984 to febraury,1987 in Los Angeless,U.S.A.73 cases of non-abused children were obtained as control.Both eyes of each case were taken and examined histo- pathologically.The intraocular changes occured in 64% of the fatally abused children.They were:Retinal hemorrhage,vitrous hemorrhage,retinal de- tachmedt,papilledema,subdural hemorrhage of the optic nerve and traum- atic cataract.On the other hand,the intraocular changes occured in 28% of control cass,especially in those of traffic and other accidents which usually caused the injuries of head and neck.Considering the intraocular changes and the general injuries together,we suggest that the most important mec- hanism of the intraocular changes be head injuries and whiplash syndrome.

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 establish an HPLC method for evaluating deoxyschizandrin in rat plasma; To explore deoxyschizandrin metabolic processes in blood.Methods The blood microdialysis probe was implanted into the right atrial enlargement through right jugular vein to detect the concentration of deoxyschizandrin in blood. Chromatographic conditions for detecting of dialysis fluid were as follows: mobile phase was methanol-acetonitrile- water (40:35:25); flow rate was 1 mL/min; column temperature was 30℃; UV detection wavelength was 254 nm. The data obtained after being calculated by the recovery rate of the probe in vivo were dealt with DAS 2.0 software. Pharmacokinetic parameters were calculated, and the concentration-time related curves were plotted.Results The deoxyschizandrin curve was linear (r=0.999 5) within the range of 2–500μg/mL. The RSD of intra-day pecision and inter-day pecision were both <3%. The stability of the solution was good in 12 hours at roomtemperature. ke was (0.364±0.047)/h; t1/2 was (1.637±0.302)h; Tmax was (0.612±0.194)h; Cmax was (165.92±28.830)μg/mL; AUC0-t was (240.793±25.540)μg?h/mL; AUC0-∞ was (282.710±30.727)μg?h/mL.Conclusion The method is convenient to perform, and the retention time of HPLC is short. This method is suitable for detecting the content of deoxyschizandrin in dialysis fluid, which can also be used to investigate the metabolic processes of deoxyschizandrin in the rat blood.