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.

p38 mitogen-activated protein kinase (MAPK) is an important intracellular signaling transduction pathway.Its major role is involved in inflammatory regulation and apoptosis.p38 MAPK is activated during the cerebral ischemia.Its expression level and the upstream and downstream protein phosphorylation levels will change.Ischemic preconditioning may mediate the survival or death of neuronal cells after cerebral ischemia through signaling transduction pathway.

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.

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.

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 evaluate the status quo and trend of the application of narcotic drugs and psychotropic substances Ⅰ in Yangzhou area.METHODS: The narcotic drugs and psychotropic substances Ⅰ used in 124 hospitals of Yangzhou from 2005 to 2007 were analyzed in respect of consumption sum,DDDs and DDC etc. RESULTS: A total of 13 kinds of narcotic drugs and 4 psychotropic substances Ⅰ were used in the 124 hospitals,and the consumption sum of these drugs increased year on year.Fentanyl remained at the first place in terms of DDDs in each year,Remifentanil Hydrochloride for Injection and Sufentanil Citrate showed an obvious increasing trend in consumption,but the consumption of Bucinnazine Hydrochloride Injection(Tablet),Triazolam and Buprenorphine Hydrochloride Injection decreased to a large extent.CONCLUSION: The use of narcotic drugs and psychotropic substances Ⅰ in the 124 hospitals in Yangzhou area was basically in line with the standard.However,the DDDs of Pethidine Hydrochloride Injection had all along dominated the front place suggesting that clinicians should attach great importance to the standard treatment of cancer pain.

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.