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 .

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.

Extensive use of tributyltin (TBT) has caused serious environmental pollution, which is harmful to human and other organisms. As a cytotoxic chemical, an important mode for TBT effects on the cells is to induce apoptosis. The present paper mainly reviewed the mechanism by which TBT induces apoptosis at low concentration.

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.

In the course of patient care, it may occur that doctors and patients have unbalanced grasp of medical information, which directly affects the building of doctor-patient trust and patients'evaluation of medical effects. Instrumental methods for building and enhancing doctor-patient trust include mainly the use of relative networks or the presentation of favors while affective methods chiefly include mutual respect and the promotion of friendly relations via communication. The rebuilding of doctor-patient relationship of trust, however, can be approached from the following seven perspectives: ①establishing and perfecting the system of rendering information accessible to the public; ②raising the managers'awareness of trust; ③building an atmosphere of trust acceptable to the entire staff members; ④accepting the restraint of standards and morality; ⑤rendering information accessible to the public in a realistic way; ⑥taking the initiative in clearing up doubts and performing the obligation of notification; and ⑦perfecting the mechanisms of supervision and reward.

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.

OBJECTIVE:To compare the efficacy and safety between caspofungin and fluconazole in the treatment of severe pulmonary fungal infection in intensive care unit. METHODS:36 patients with systemic fungal infections in intensive care unit were randomly assigned to receive caspofungin 50 mg?d-1 which was added into 250 mL normal saline and infused in no less than 1h for 14 days after initial dose of 70 mg(caspofungin group,n=18) or fluconazole 400 mg?d-1 for 14 days by iv gtt (fluconazole group,n=18). The curative efficacy and adverse events in the two groups were observed. RESULTS:The effective rate was 100.0% in caspofungin group versus 66.7% in fluconazole group. The incidence rates were 22.2% and 72.2%,respectively. There were significant differences in the overall effective rate between two groups(P

We detected varicella-zoster virus (VZV) DNA sequences in bullous lesions and skin biopsies obtained from healed skin lesions in 16 patients with herpes zoster using polymerase chain reaction. A 385 bp VZV DNA fragment was found in all the bullous lesions and in two of six biopsies from the skin lesions healed within two months. No VZV DNA was found in the skin lesions two months after healing in 10 cases of herpes zoster. VZV DNA may be detected at the sites of resolved herpes zoster lesions within short duration.