Objective To summarize the clinical effect of Doty procedure on surgical treatment for patients with congenital supravalvular aortic stenosis (SVAS).Methods Between Jan.2009 and Jun.2013,12 cases of consecutive patients underwent surgical corrections of SVAS using Doty procedure.Among them,Williams' syndrome were present in 5 patients.Nine cases were male,and 3 cases were female.Mean age was(5.3 ±4.2) years(9 months to 12 years) and mean weight was (11.5 ± 5.3) kg (7.5-32.0 kg).Echocardiography and Computed Tomography confirmed the diagnosis.SVAS was discrete in 9 patients and diffuse in 3 patients.Mean peak preoperative gradient was 6.86-16.23 (11.07± 3.68) kPa,with 3 patients above 13.33 kPa.Bicuspid aortic valve was present in 2 patients,mild aortic valve stenosis in 1 patient,mild aortic regurgitation in 2 patients,1 patient with subaortic membrane,3 patients with stenosis of the left or the right pulmonary artery,1 patient with pulmonary valve stenosis,2 patients with PDA,moderate to severe mitral insufficiency in 1 patients.Surgery was performed using Doty procedure through median sternotomy with cardiopulmonary bypass.Results Mean time of cardiopulmonary bypass was (85 ± 26)min (68-129 min).Mean time of aortic clamp was (51 ± 16) min(43-68 min).There was no early death and low cardiac output syndrome.Mild ST segment change was present in 5 patients and occasional premature beat was in 3 patients.Ventilator was applied for 7 to 38 h and Cardiac Intensive Care Unit treatment was done for 1 to 4 d.The mean peak supravalvular gradient measured postoperatively was (2.18 ± 1.09) kPa.Follow-up data were available for 11 patients,ranged from 6 months to 3 years.The gradients across supravalvular were lower than 3.33 kPa,except for 2 patients with severe SVAS preoperatively.There was no late death,dizzy or palpitation during follow-up.Conclusion Doty produce for SVAS can get satisfactory clinical results.

Aged ; Female ; Humans ; Laminectomy ; methods ; Lumbar Vertebrae ; surgery ; Male ; Middle Aged ; Spinal Canal ; surgery ; Spinal Stenosis ; surgery ; Treatment Outcome

Objective To evaluate the efficacy of Lianhua Dingchuan Tablets in bronchial asthma.Methods Fifty BALB/C mice were randomly and equally divided into control (Con) group,ovalbumin (OVA) group,dexamethasone (DEX) group,high-dose Lianhua group,low-dose Lianhua group.The mice were sensitized and challenged with OVA plus aluminium hydroxide to establish asthmatic model and were pre-treated 30 minutes before challenge.Specific airway resistance (sRaw) was used to evaluate airway hyperresponsiveness,and airway inflammatory changes were measured.ELISA and Magnetic Luminex(R) were used to quantified the levels of IL-4,IL-13 and INF-γ.Results Airway resistance significantly decreased in DEX group and High-dose Lianhua group (P＜0.05).Levels of inflammatory cells and IL-13 in BALF evidently reduced in DEX group,high-dose Lianhua group and low-dose Lianhua group (P ＜ 0.05),while IL-13 level in serum only decreased in DEX group.There was no significant changes in the levels of IL 4 and INF γ among those groups.Conclusions Lianhua Dingchuan Tablets might relieve the symptoms of asthma by reducing IL-13 level and inhibiting the airway inflammation.

We constructed different N-terminal truncated variants based on Bacillus acidopullulyticus pullulanase 3D structure (PDB code 2WAN), and studied the effects of truncated mutation on soluble expression, enzymatic properties, and application in saccharification. Upon expression, the variants of X45 domain deletion existed as inclusion bodies, whereas deletion of CBM41 domain had an effective effect on soluble expression level. The variants that lack of CBM41 (M1), lack of X25 (M3), and lack both of CBM41 and X25 (M5) had the same optimal pH (5.0) and optimal temperature (60 degrees C) with the wild-type pullulanase (WT). The K(m) of M1 and M5 were 1.42 mg/mL and 1.85 mg/mL, respectively, 2.4- and 3.1-fold higher than that of the WT. k(cat)/K(m) value of M5 was 40% lower than that of the WT. Substrate specificity results show that the enzymes exhibited greater activity with the low-molecular-weight dextrin than with high-molecular-weight soluble starch. When pullulanases were added to the saccharification reaction system, the dextrose equivalent of the WT, M1, M3, and M5 were 93.6%, 94.7%, 94.5%, and93.1%, respectively. These results indicate that the deletion of CBM41 domain and/or X25 domain did not affect the practical application in starch saccharification process. Furthermore, low-molecular-weight variants facilitate the heterologous expression. Truncated variants may be more suitable for industrial production than the WT.
Bacillus ; enzymology ; Glycoside Hydrolases ; metabolism ; Molecular Weight ; Protein Conformation ; Sequence Deletion ; Substrate Specificity ; Temperature

OBJECTIVETo explore the protective effect of baicalin against rotenone-induced injury on PC12 cells, and the po-tential mechanism of action action was also explored.

METHODPC12 cells were injured by rotenone and were treated with different concentrations (0.1, 1, 10 μmol x L(-1)) of baicalin at the same time. Cell viability was analyzed by MTT, and morphology was observed by phase-contrast microscopy. The cell apoptosis was detected by flow cytometry by Annexin V-FITC/PI staining. The intracellular ROS level was determined by fluorescence microscope with DCF-DA staining. The expression of Bcl-2, Bax and Caspase-3 was analyzed by Western blot.

RESULTThe viability of PC12 cells exposure to rotenone for 24 hour was gradually decreased with dose escalating and 1.5 μmol x L was adopted to do the following experiment. Baicalin increased cell viability, improved cell morphology and decreased intracellular ROS level. Moreover, FACS indicated baicalin attenuated the apoptosis induced by rotenone significantly. Western blot showed that Bcl-2, Bax and Caspase-3 expression in rotenone-induced PC12 cells was reversed by baicalin.

CONCLUSIONThis study has demonstrated that baicalin protects PC12 cells against rotenone-induced apoptosis, at least in part, by scavenging excessive ROS and inhibiting the mitochondrion-dependent apoptotic pathway.

Animals ; Apoptosis ; drug effects ; Caspase 3 ; metabolism ; Cell Survival ; drug effects ; Cytoprotection ; drug effects ; Flavonoids ; pharmacology ; Gene Expression Regulation ; drug effects ; Intracellular Space ; drug effects ; metabolism ; PC12 Cells ; Proto-Oncogene Proteins c-bcl-2 ; metabolism ; Rats ; Reactive Oxygen Species ; metabolism ; Rotenone ; pharmacology ; bcl-2-Associated X Protein ; metabolism

Objective To judge method performance of routine biochemistry parameters on Roche Modular PPI testing system by using westgard's method evaluation decision chart.Methods We assessed imprecision(CV)from internal quality control and inaccuracy(bias)from external quality control evaluation.Combined estimates of imprecision and inaccuracy by plotting imprecision as the x-coordinate and inaccuracy as the y-coordinate to locate an expected operating point of every item on the chart.By comparing this operating point with allowable total errors(TEa),we can decide whether the performance is acceptable or not.Results In the 27 different parameters tested,imprecision and bias of calcium were 0.08 mmol/L and 0.06 mmol/L respectively,its performance was marginal.The imprecision of creatinine,urea,glucose, sodium,chloride and phosphorus were 3.20%,2.13%,1.52%,0.89 mmol/L,1.10% and 1.55%,the bias were 4.79%,0.96%,4.63%,0.80 mmol/L,1.74% and 4.13% respectively,their performance was good.M1 other 20 items were of excellence performance.Conclusions Routine biochemistry parameters on Roche Modular PPI testing system possessed good precision and accuracy,and their performance were acceptable.To judge method performance of biochemistry testing system by using westgard' s method evaluation decision chart was easy to do and suited for clinical laboratory.

Background Congenital heart disease (CHD) is a diverse group of diseases determined by genetic and environmental factors.Considerable research has been done on genes associated with the development of the heart.Recently,focus is on the role of transcription factor NFATc1 in the development of proper valve and septa.As part of a larger study,high density single nucleotide polymorphism (SNP) scanning was used to explore the relationship between NFATc1 gene polymorphism and susceptibility to ventricular septal defect (VSD) in the Chinese Han population.Methods One hundred and ninety-two pediatric patients with congenital VSD and 192 matching healthy control subjects were studied.The haplotype reconstructions were calculated by PHASE2.0 software.Haploview software was used to perform linkage disequilibrium assessment and define haplotype blocks.The algorithm used for defining the blocks was the confidence interval method.Results The NFATc1 gene region can be divided into 11 haplotype blocks.Strong linkage disequilibrium existed within blocks 6,8,9,and 11.Three SNPs (rs7240256,rs11665469,and rs754505) within the NFATc1 gene had significant correlation with VSD by single marker association analysis.In addition,two haplotypes correlated with VSD.Conclusions NFATc1 is associated with the occurrence of VSD and it may be a predisposing gene to CHD in Han Chinese.This finding has set a direction for further genetic and functional studies.

Objective: To analyze the clinical features and surgical treatment of unroofed coronary sinus syndrome(UCSS) in children, and to provide evidence for the diagnosis and treatment of such diseases during the perio-perative period. Methods: A retrospective analysis was conducted based on the clinical data of 13 patients with UCSS, who underwent surgical treatment at Beijing Children′s Hospital Affiliated to Capital Medical University from January 2011 to December 2017.All 13 patients were complicated with persistent left superior vena cava(PLSVC) and other cardiac malformations; 7 patients were diagnosed preoperatively and 6 patients were diagnosed intraoperatively.Eleven cases were diagnosed type Ⅰ and 2 cases were type Ⅳ according to Kirklin′s classification.In all of 13 cases, atrial septal reconstruction was performed to drain the left superior vena cava(LSVC) to the right atrium, and other cardiac malformations were corrected at the same time. Results: One patient died in this group, and the other 12 patients had early recovery after operation.Postoperative-ventilator-assisted time was 5-246 h(median 29 h) and hospital stay time was 8-40 d(median 16 d). The remaining 12 cases were followed up from 6 months to 7 years and 2 months.No death or complications occurred.No residual shunt or residual obstruction was found. Conclusions In the case of congenital heart disease with PLSVC, UCSS should be alerted.Atrial septal reconstruction and drainage of LSVC into right atrium in children with UCSS can achieve satisfactory surgical results.

BACKGROUNDVascular rings are uncommon anomalies in which preferred strategies for diagnosis and management may vary among institutions. In this study, we reported our approach and a review of our 5-year experience.

METHODSFrom May 2006 to April 2011, 45 children (31 boys) with vascular rings underwent surgical repair at Beijing Children's Hospital. Nineteen patients (26%) had associated heart anomalies.

RESULTSThere were two hospital deaths. At follow-up, 11 patients still had intermittent respiratory symptoms, but these symptoms had no effect on growth or physical activities. No patients required reoperation.

CONCLUSIONSThe rates of misdiagnosis and missed diagnosis of vascular rings are higher than those of other congenital heart diseases. A high index of clinical suspicion coupled with the use of computed tomography enables early diagnosis. Surgical repair can be performed successfully, although a number of patients will have persistent symptoms.

Child, Preschool ; Female ; Humans ; Infant ; Male ; Tracheal Stenosis ; mortality ; surgery ; Vascular Malformations ; diagnosis ; mortality ; surgery

BACKGROUNDThe shape memory effect of nickel-titanium (NiTi) archwires is largely determined by the phase transition temperature. It is associated with a reversible transformation from martensite to austenite. The aim of this study was to characterize austenite, martensite and R phase temperatures as well as transition temperature ranges of the commonly used clinical NiTi orthodontic arch wires selected from several manufacturers.

METHODSDifferential scanning calorimetry (DSC) method was used to study the phase transformation temperatures and the phase transition processes of 9 commonly used clinical NiTi alloys (types: 0.40 mm (0.016 inch), 0.40 mm x 0.56 mm (0.016 inch x 0.022 inch)).

RESULTSThe austenite finish temperatures (Af) of 0.40 mm Smart, Ormco and 3M NiTi wires were lower than the room temperature, and no phase transformation was detected during oral temperature. Therefore, we predicted that these types of NiTi did not possess shape memory property. For 0.40 mm and 0.40 mm x 0.56 mm Youyan I NiTi wires, no phase transformation was detected during the scanning temperature range, suggesting that these two types of wires did not possess shape memory either. The Af of 0.40 mm x 0.56 mm Smart, L&H, Youyan II Ni-Ti wires were close to the oral temperature and presented as martensitic-austenitic structures at room temperature, suggesting the NiTi wires listed above have good shape memory effect. Although the 0.40 mm x 0.56 mm Damon CuNiTi wire showed martensitic-austenitic structures at oral temperature, its Af was much higher than the oral temperature. It means that transformation from martensite to austenite for this type of NiTi only finishes when oral temperature is above normal.

CONCLUSIONThe phase transformation temperatures and transformation behavior varied among different commonly used NiTi orthodontic arch wires, leading to variability in shape memory effect.

Calorimetry, Differential Scanning ; Dental Alloys ; chemistry ; Nickel ; chemistry ; Orthodontic Wires ; Temperature ; Titanium ; chemistry