Objective To explore the long-term prognosis of the patientswithpulmonary arterial hypertension ( PAH) after cardiac repair operations through follow up .Methods We filtered the CHD patients with PAH after cardiac repair operation , admitted to the department of pediatric cardiology in Beijing Anzhen Hospital from June 2004 to December 2014, and collected their preoperative and postoperative data to analysis the long-term prognosis.Results 92 patients were included, median age was 20.78 years(2.44-60.26 years),median operation age was 13.54 years(1.33-53.99 years), World Health Organiza-tion functional classⅠ-Ⅱ was 80(86.96%), Ⅲ -Ⅳ was 12(13.04%), mean 6 min walk test distance was(481.74 ± 80.74) meters, mean Borg score was 1.53 ±1.14, and mean percutaneous oxygen saturation was 0.95 ±0.05.Among them, 64 patients completed preoperative right heart catheterization.Their average pulmonary artery pressure was(72.23 ±12.83) mmHg, aortic pressure on average was(85.57 ±11.91) mmHg, and average pulmonary vascular resistancewas(10.79 ±5. 04) Wood· U.The range of follow-up was between 6 months and 28.32 years(median 13.67years).During the follow-up, there were arrhythmia in 2 cases(2.17%), syncope in 7 cases(7.61%), hemoptysis in 4 cases(4.35%),heart failure in 8 cases(8.70%) and death in 1 case(1.09%).Compared with preoperative data, WHO cardiac function was found decreased in 22 patients(23.91%), stabled in 61 patients(66.30%) and significantly improved in 9 patients (9.78%).63 patients (68.48%) received targeted drug therapy, only 5 patients were treated with combined therapy,29 cases were not.Conclusion Compared with idiopathic pulmonary hypertension , PAH after defect correction has a lower incidence of arrhythmia , hemop-tysis and heart failure, and a higher long-term survival rate, and heart function is stable.To patients with PAH after defect cor-rection, targeted therapy is not only in perioperative period, but also more important in the long-term after surgery.In the targe-ted drug therapy, the combination treatment rate is low.

Objective To explore the risk factors in disease progression and the significance of vascular endothelial growth factor ( VEGF ) and vascular intercellular adhesion molecule-1 ( VCAM-1 ) in hand foot and mouth disease ( HFMD) combined with encephalitis .Methods Altogether 92 subjects with HFMD were enrolled in the study and were divided into four groups , including group A ( ordinary group with no complication ) , group B ( severe group with complication ) , group C ( critical group with complication ) , group D ( recovery group with complication ) .Concentrations of VEGF and VCAM-1 in serum and cerebro-spinal fluid were detected by double antibody sandwich ELISA method .Multiple factors logistic regression analysis was performed to analyze main risk factors in disease progression for two combinations , one for ordi-nary group and severe group , the other for severe group and critical group .The results were analyzed by SPSS16.0 statistical software.Results The concentration of VEGF and VCAM-1 in serum and cerebrospi-nal fluid had statistically significant differences among the four groups , but there was no significant difference between group A and group D , and between group B and group C .In addition , the statistically significant factors for prediction of disease progression were duration of fever , limb shaking, cerebrospinal fluid WBC , cerebrospinal fluid protein and EV 71 IgM between ordinary group and severe group , and cerebrospinal fluid WBC, respiratory rate and heart rate between severe group and critical group .The multiple factors logistic regression analysis revealed that limb shaking , cerebrospinal fluid protein , VEGF and VCAM-1 in serum were the main risk factors for disease progression from ordinary to severe (P=0.071, 0.019, 0.020, 0.025 and OR=147.629, 26.572, 5.958, 6.345).And increased heart rate indicated the progression from se-vere to critical with P value of 0.001 and OR value of 2.69.Conclusion (1) Compared with group A, VEGF and VCAM-1 in serum and cerebrospinal fluid were highly expressed in patients with HFMD combined with encephalitis .Therefore , VEGF and VCAM-1 could be used as diagnostic criteria for auxiliary diagnosis of encephalitis in patients with HFMD and reflect the severity and prognosis to a certain extent .( 2 ) Risk factors like limb shaking , cerebrospinal fluid protein , VEGF and VCAM-1 in serum would be helpful to early diagnosis of severe patients .Increased heart rate would be a significant factor for identification of patients with critical disease , according to which a timely treatment would be provided to prevent from worse .

Objective: To analyze the prognosis and associated risk factors of pediatric idiopathic pulmonary arterial hypertension. Methods: A total of 119 patients under 18 years of age diagnosed as idiopathic pulmonary arterial hypertension in the Pulmonary Arterial Hypertension Center in Beijing Anzhen Hospital between June 2007 and May 2017 were enrolled in this retrospective study. The clinical informations and follow-up data were collected. The endpoints of follow-up were defined as death or undergoing lung transplantation. Kaplan-Meier survival curve was used to assess the survival,and the COX risk regression model was used to analyze the prognostic risk factors. Results: The mean age at diagnosis was (5.9±4.2) years. For 92 (77.5%) patients, the main reason for visit was decreased activity with shortness of breath after exercise. Seventy patients (58.8%) were in baseline NYHA functional class Ⅲ-Ⅳ and 49 patients (41.2%) were in NYHA functional class Ⅰ-Ⅱ. The mean systolic pulmonary arterial pressure estimated by echocardiography was (90±23) mmHg (1 mmHg=0.133 kPa) . Right heart catheterization was performed in 50 patients. Hemodynamic parameters revealed that the mean pulmonary artery pressure was (66±19) mmHg. Mean right atrium pressure was (8.5±3.4) mmHg. Mean pulmonary vascular resistance index was (17±9) wood·m² and the mean cardiac index was (3.4±1.3)L/m²; 100 patients (84.0%) received targeted therapy in which 55 patients (46.2%) were on monotherapy,40 patients (33.6%) were on dual therapy and 5 patients (4.2%) were on triple therapy. The mean time of follow-up was 22.0 months (0-108 months). During follow-up, 43 patients (36.1%) died and 1 patient received double-lung transplantation. Main causes of death including right heart failure, pulmonary hypertension crisis, asphyxia and massive hemoptysis. The mean survival time from diagnosis was 37.0 months,1-,2-,3-and 5-year survival rates were 86.3%, 72.2%, 51.4%and 37.8% respectively. Survival analysis showed that patients in baseline NYHA functional class Ⅰ-Ⅱ had better prognosis. COX regression analysis showed that NYHA function class, edema, increased total bilirubin and troponin concentration and the pulmonary artery and aorta diameter ratio measured by echocardiogram are risk factors of pediatric IPAH (HR=2.310, 2.723, 1.066, 1.696, 3.719, P=0.028, 0.005, 0.001, 0.024, 0.030) . While the existence of aterial septal defect or patent foramen ovale, using bosentan and phosphodiesterase inhibitors_, dual or triple therapy were protective factors (HR=0.563, 0.559, 0.603, 0.682, 0.044, P=0.169, 0.076, 0.115, 0.258, 0.220) . In multivariate analysis only edema associated with decreased survival (HR=2.398, P=0.025) . Conclusion Childhood idiopathic pulmonary arterial hypertension patients are seriously ill at visit. Worse cardiac function classification at visit associate with high mortality. Target therapy including using bosentan, dual or triple therapy can improve survival.

Inositol 1,4,5-trisphosphate receptor 1 (ITPR1) is an important intracellular channel for releasing Ca²⁺. In order to investigate the effects of the ITPR1 overexpression on Ca²⁺ concentration and lipid content in duck uterine epithelial cells and its effects on calcium transport-related genes, the structural domain of ITPR1 gene of duck was cloned into an eukaryotic expression vector and transfected into duck uterine epithelial cells. The overexpression of the ITPR1 gene, the concentration of Ca²⁺, the lipid content, and the expression of other 6 calcium transport-related genes was determined. The results showed that the concentration of Ca²⁺ in uterine epithelial cells was significantly reduced after transfection (P<0.05), the triglyceride content was significantly increased (P<0.01), and the high-density lipoprotein content was significantly decreased (P<0.01). The correlation analysis results showed that the overexpression of the C-terminal half of the ITPR1 gene was significantly positively correlated with the total cholesterol content (P<0.01), which was significantly positively correlated with the low-density lipoprotein content (P<0.05). The overexpression of the N-terminal half of the ITPR1 gene was significantly positively correlated with the triglyceride content (P<0.01), which was significantly negatively correlated with the concentration of Ca²⁺ (P<0.05). RT-qPCR results of 6 calcium transport-related genes showed that the overexpression of the C-terminal half of the ITPR1 gene significantly inhibited the expression of the IP3R2, VDAC2 and CAV1 genes, and the overexpression of the N-terminal half of the ITPR1 gene significantly promoted the expression of the IP3R3 and CACNA2D1 genes. In conclusion, the ITPR1 gene overexpression can promote Ca²⁺ release in duck uterus epithelial cells, promote the synthesis of triglyceride, low-density lipoprotein and cholesterol, and inhibit the production of high-density lipoprotein, and the ITPR1 gene overexpression affected the expression of all 6 calcium transport-related genes.
Animals ; Calcium/metabolism* ; Ducks/genetics* ; Epithelial Cells ; Female ; Inositol ; Inositol 1,4,5-Trisphosphate Receptors ; Lipids ; Uterus