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.

Child, Preschool ; Humans ; Influenza A Virus, H1N1 Subtype ; Influenza, Human ; virology ; Male

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.

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.

Objective To observe the effect of laser irradiation on the level of histamine (Hm) , 5- hydroxytryptamine(5-HT), interleukin-1 (IL-1), tumor necrosis factor α (TNF-α) in serum and β- endorphin (β- EP) in brain tissue in rat model of rheumatoid arthritis. Methods A total of 60 Wistar rats were randomly divided into four groups:a normal group, a model group (RA without treatment), a laser + drug treatment group (RA treated by laser and drug) and a drug treatment group (RA treated by drug alone). All the groups, except the normal one, were injected with complete Freund's adjuvant to make animal model of adjuvant arthritis. All the animals were then treated accordingly. The level of Hm, 5-HT, IL-1 and TNFα in serum and β-EP in brain tissue were measured. Results Laser downregulated the content of Hm, 5-HT, IL-1 and TNFα in serum and increased the content of β-EP in brain tissue. Conclusion Laser irradiation is an effective modality for treating the animal model of rheumatoid arthritis.

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 investigate the clinical value of computed radiography(CR),computed tomography(CT)and magnetic resonance imaging(MRI)in spinal tuberculosis.[Method]Forty-one patients with histologically confirmed spinal tuberculosis underwant imaging examinations.Forty-one patients were performed CR,22 CT and 31 MRI.[Result]CR of 38 patients clearly revealed bone changes and narrowed or disappeared disk space.CR of 30 patients well showed the abnormal soft tissue.CT scan of 22 patients clearly depicted calcification and no abnormal was found in pedicles in all the 22 CT scans.CT scan of 21 patients revealed bone changes of vertebral bodies and CT scan of 20 patients showed abnormal soft tissue.The soft tissue was involved in all 31 patients with spinal tuberculosis.Diskspaces were involved in 29 cases and the pedicles were normal in all 31 case.The lesions showed hypointense in T1 scan primarily,hyperintense in T2 scan primarily.[Conclusion]CR is the first imaging approach for spinal tuberculosis.CT well reveal the extent and minute calcification.The extent,abnormal soft-tissue and abnormal disk space are more dramatically demonstrated on MRI imaging.CR,in addition to CT and MRI,is helpful in diagnosis of spinaltubereuinsis.