Bicuspid aortic valve(BAV) is one of the most common congenital cardiovascular malformations,affecting 1% to 2% of the population.BAV may be sporadic or familial.BAV is likely a multiple genetic disorder based on the study of heritability and identification of pathogenic genes and potential candidate genes.With increasing studies of related genes of BAV,early intervention and gene therapy of BAV and associated complications may be likely in the near future.

Child ; Humans ; Tachycardia ; physiopathology ; therapy

PAX2 gene encodes a nuclear transcription factor and plays a pivotal role for embryonic kidney development.It is expressed during the entire developmental process of pronephron,mesonephron and metanephron,involving in the regulation of embryonic kidney development at various fetal stages,but its expression disappeared in mature nephrons.Recently,it was found that PAX2 was re-expressed in nephropathy.Gene mutation and re-expression of PAX2 are closely related with congenital and acquired renal diseases.This paper reviews the role and significance of PAX2 expression in kidney disease from the aspects of normal expression,gene mutation and abnormal expression.

Objective To evaluate the recovery and hemedynamics of total intravenous anaesthesia (TIVA) with propofol and remifentanil. Methods Thirty ASA Ⅰ - Ⅱ patients undergoing hperoscopic oper-ation were randomly divided into the TIVA group(group A,n=15) and the combined intravenous with inhala-tion anaesthesia greup(group B,n=15). Anaesthesia was induced in the same manner in both greups:Midazo-lam 0.05mg/kg,vecurium 0.1mg/kg,propofol 1.5mg/kg and remifentanil 1.5 μg/kg.Remifentanil was infused 2.5 μg/ml) in group A or with sevoflurane( 1.3% end-tidal) in group B to maintain anaesthesia. With termi-nation of surgery,anaesthetic delivery was discontinued simuhaneously.Bp,HR at all stages and recovery profiles were recorded. Results (1)Between before inflation and after inflation or between at the end of op-eration and the basic values in group A,there were no significant differences in Bp,HR. (2) Times from stop-ping administration of anaesthetics until full spontaneous respiration and eye opening were similar for two groups.Time for trachcal extubation was significantly less in the group A than group B. (3)The rates of post-operative nausea or vomiting were similar. Conclusion The application of TIVA with propefol and remifen-tanil is associated with better hemodynamic stability and better recovery profile as compared to the combined intravenous with inhalation anaesthesia.

ts The presence of +276G allele was significantly higher in the study group as compared with the control group.Conclusion Subjects with the +276G variant alleles may have a greater risk for weight gain after antipsychotic treatment.

Objective To evaluate the uniformity of different diagnosis criteria of metabolic syn-drome in patients with type 2 diabetes, especially the criteria that suit for metabolic syndrome in type 2 dia-betes patients.Methods The patients with type 2 diabetes, aged from 20~74 years, who were treated at Department of Endocrinology , the Third Affiliated Hospital , Qiqihar Medical University from January 2008 to November 2009 were enrolled.Four hundred and twenty one patients with integral data were analyzed . Waistline, hip line, height, body mass, blood pressure and so on were surveyed , and according to waistline and hip line the waist-to-hip ratio was calculated .On the basis of body mase/height body mass index ( kg/m2 ) was calculated .The blood sugar was detected with the hexokinase method .The blood cholesterol , tria-cylglycerol , the high density lipoprotein cholesterol were determined with the biochemistry method .Urinates the micro albumin level was determined with chemistry to try the belt technology .Diagnosis standards of WHO (1999), NCEP-ATPⅢ(2001),ACCE(2003),EGIR(1999),the Chinese CDS(2007) suggestion metabolism synthesis drafts , the triacylglycerol-waistline standard of Blackburn P forecast cardiovascular e-vent(2003) were applied to calculate prevalence rate of metabolism synthesis .Results The 421 enrolled testees were involved in the result analysis .⑴Prevalence rates of type 2diabetes and metabolism synthesis in various single component: In the investigation population the differences in body of obesity , hypertension and hypertriacylglycerolemia , the low and high density lipoprotein cholesterol blood sickness between the men and women were insignificant , but the overload (66.1%) in males was remarkably higher than that in females (49.7%).The prevalence rate in abdomen obese in males (74.0%) was remarkably higher than that in females(59.9%).⑵Abnormal distribution of metabolism in patients with type 2 diabetes: Preva-lence rate of one kind of abnormal metabolism was 27.2%.Prevalence rate of two kinds of or above two kinds of above abnormal metabolism was 61.0%.Prevalence rate of three kinds of above abnormal metabo-lism was 26.1%,and the prevalence rate in females was not higher than that of males ( P >0.05 ) .⑶After age and sex lamination ,prevalence rates of metabolism synthesis and it's the correlated disease:The preva-lence rates of the high hypertriacylglycerolemia , hypertension , metabolism synthesis had the tendency of in-creasing along with the age .Prevalence rates of abdominal obesity and the low and high density lipoprotein cholesterol blood sickness had the increased tendency in females , but it was not obvious in males .Except that 35~44 years old of age group prevalence rate of the high hypertriacylglycerolemia in males ( 62.1%) was remarkably higher than that in females ( 29.2%) ( P <0.005 ) , and the 20~35 years old age group prevalence rates of metabolism synthesis in males (41.2%)were remarkably higher than outside the females (20.0%)( P <0.05).In other various age groups ,there was insignificant difference in prevalence rate between males and females .⑷The prevalence rate of WHO diagnosis standard for metabolism synthesis (62.9%) had good concordance with the prevalence rate of the Chinese CDS diagnosis standard for metabo -lism-synthesis ( 66.7%) .The prevalence rate of ACCE diagnosis standard for metabolism synthesis (88.8%) had good concordance with the prevalence rate of the EGIR diagnosis standard for metabolism synthesis (86.6%).The prevalence rate of Blackburn P forecast cardiovascular event (20.9%),which was distanced from the result of the diagnosis standards ( P <0.005 ) .Conclusion ⑴Patients with type 2 di-abetes have high metabolism syndrome proportion rate , and the abnormal metabolism proportion rate in sin-gle group is also higher.⑵WHO (1999) metabolism syndrome diagnosis standard and CDS (2007)sugges-tion standard are suitable for Chinese metabolism -synthesis in type 2 diabetic patients .⑶The metabolism syndrome diagnostic standards of EGIR and ACCE are similar in proportion rate in Chinese patients with type 2 diabetes , which is accorded with the findings of former literatures .Whether that can be used widely deserves further study .⑷The diagnostic criteria of triacylglycerol waistline is not suitable for Chinese type 2diabetic patients.

Objective To estimate direct economic burden of patients with moderate to severe psoriasis in China,and to assess its influencing factors.Methods A questionnaire survey was carried out in 150 patients with moderate to severe psoriasis from 29 third-grade class-A hospitals in 7 cities.Data on demographic characteristics,clinical features and direct economic burden were collected.Descriptive statistics were performed to analyze the direct economic burden of moderate to severe psoriasis,and multiple regression analysis was carried out to determine predictors of cost of illness.Results The patients were aged (43.87 ± 13.42) years,with an average clinical course of 3 years(range,2-6 years).Of them,82 had moderate psoriasis,and 68 had severe psoriasis.The average direct cost was (6 452 ± 6 391)RMB per patient-year,with significant difference between the patients with moderate psoriasis and those with severe psoriasis ((4 163 ± 4 605) vs.(9 212 ± 7 146) RMB,z =5.70,P ＜ 0.001).Regression analysis indicated that disease severity,clinical type,hospitalization,complications,and inhabited cities were factors influencing the direct economic burden of patients with moderate to severe psoriasis.Conclusions The direct eonomic burden of severe psoriasis seems to be twice that of moderate psoriasis in China.Physicians should strengthen the control of complications and disease severity,and pay more attention to other types of psoriasis than psoriasis vulgaris,during the treatment of psoriasis.

Objective To diagnose retinal detachment with International Classification of Diseases10 (ICD-10) and to analyze the surgical modalities of vitrectomy for the disease.Methods The clinical data of 1 648 hospitalized patients with retinal detachment undergoing vitrectomy in our hospital in 2013 were retrospectively analyzed.Results The ICD etiological classification showed that primary retinal detachment accounted for 50.18％ (827/1 648),tractional retinal detachment accounted for 32.52％ (536/1 648),exudative retinal detachment accounted for 0.61％ (10/1 648)and other types accounted for 16.69％ (275/ 1 648).The top 5 common etiological diagnoses were simple rhegrnatogenous retinal detachment (25.73％,424/1 648),diabetic tractional retinal detachment (12.62％,208/1 648),traumatic retinal detachment (12.08％,199/1 648),other primary retinal detachment (11.23％,185/1 648) and retinal detachment after intraocular surgery (10.62％,175/1 648).The top 4 modalities of vitrectomy were combined silicone oil implanting surgery (54.73％,902/1 648),basic vitrectomy (21.97％,362/1 648),combined silicone oil and lenticular surgery (14.38％,237/1 648) and combined lenticular surgery (5.58％,92/1 648).Conclusions Modern vitrectomy is used mainly for the treatment of primary retinal detachment and the treatment of retinal detachment related to diabetes,ocular trauma and intraocular surgeries.The complexity of various retinal detachment diseases requires different surgical modalities of vitrectomy for treatment.

Objective To evaluate the curative effect of laparoscopic Palomo operation compared with open inguinal varicocelectomy in children with varicocele, and testis development after treatment. Methods Data of 63 boys with varicocele were reviewed. The control group(n = 29) was underwent open inguinal varicocelectomy,the lap-aroscopy group(n =34) was underwent laparoscopic Palomo operation. The operation time,hospital stay,recurrence rate,and complications rate were compared between two groups. The boys in larparoscopy group were examined the diameter of spermatic vein and testicular volume before and after operation by ultrasound. All data were analyzed by SPSS 13.0 software. Results The hospital stays and recurrence rates and complications rates were significantly different between two groups (P < 0.05 ) , but the operation time in two groups had no statisticd difference (P > 0. 05 ). The diameters of spermatic vein in larparoscopy group were decreased significantly after operation (P < 0.01). The blood supply for testis didnt decrease after operation,the operative lateral testicular volume grew faster than the contra lateral,and reached almost the same volume. Conclusions Laparoscopic Paloma varicoelectomy was safe and simple, brings a better effect for children with varicocele than open inguinal varicocelectomy does. The operative lateral testis would a postoperative compensatory growth,no testis atrophy or hypogonadism occurs.

Objective To explore the clinical application of reconstructing silicone elastomer nose prosthesis by means of selected laser sintering and wax powder PCPI. Methods Laser scanning was used to get the 3-D data of a nose model. Surfacere 10.0 etc softwares was used to reconstruct the nose by mirroring the digitalized model of absent nose. Selective laser sintering and wax powder was chosen to fabricate a wax nose model and the nose prosthesis made by silicone clastomer. Results Perfect silicone clastomer nose prosthesis was made for 2 patients. Conclusion This study suggests that the wax nose model and the new wax powder can meet the requirement of clinical expectation for maxillofacial prosthesis.