The occurrence rate of long QT syndrome is 1/2 000,which accounts for approximately 10％ of cases of the sudden infant death syndrome.The morbidity is decreased after receiving appropriate therapy in early stage.Electrocardiogram (ECG) screening,with reliable results,is simple and feasible in infancy and can be used to conduct appropriate genetic testing.Neonatal screening is controversial because of individual differences in ECG results.Even there are some defects,it is still necessary to perform neonatal ECG screening which can improve prognosis in children with long QT syndrome.

Objective· To investigate whether vitamin E could reverse the disruptive effects of bisphenol A (BPA) on steroidogenesis and to explore the optimal vitamin E concentration.Methods· Rat primary granulosa cells were extracted and exposed to BPA (0,0.01,0.1,1,10,100 μmol/L).After 48 h of incubation,culture medium was collected and estradiol (E2) and progesterone (P4) were measured using ELISA kits.Then,granulosa cells were incubated with 5 μmol/L(average concentration in follicular fluid) or 25 μmol/L (high concentration in follicular fluid) vitamin E (α-tocopherol) or vitamin E (5 μmol/Lor 25 μmol/L) plus BPA (10 μmol/L or 100 μmol/L) for 48h,E2 and P4 were measured.Results· BPA at 10 μmol/L and 100 μmol/L suppressed E2 and P4 production in a dose-dependent manner (P＜0.05).Vitamin E at 25 μmol/L significantly increased E2 and P4 levels by (44.89±15.18) ％ and (43.33±8.82) ％(P＜0.05),respectively.Coincubation of the granulosa cells with BPA and vitamin E (5 μmol/L or 25 μmol/L) restored the productions of E2 and P4,which were not significantly different from the control group (P＞0.05).Conclusion· Vitamin E (5 μmol/L/25 μmol/L) could reverse BPA-induced reduction in steroid hormone production in rat ovary granulosa cells,and the antagonistic effect of 25 μmol/L vitamin E was more obvious than that of 5 μmol/L vitamin E.

Objective To study the methylation status of the promoter region of several tumor suppressor genes in p53-Bax mitochondrial apoptosis pathway and its role in cholangiocarcinoma. Methods The hypermethylation of the promoter region of tumor suppressors death-associated protein kinase (DAPK), p14, and target of methylation-associoted silencing-1 (TMS1/ASC) were detected by methylation-specific PCR. P53 gene status (exon 5-8 ) were examined by automated sequencing. The relationship between gene mutations and the biological behaviors of cholangiocarcinoma was analyzed. Results Methylation existed in at least one promoter region of tumor suppressor gene in the tumor tissues of 24 patients (66. 7% ). The frequencies of tumor suppressor gene methylation in cholangiocarcinoma were: p14 24%, DAPK 30. 6%, and TMS1/ASC 36. 1%. The frequencies of tumor suppressor gene methylation in the adjacent tissues were: TMS1/ASC 8.3% and DAPK 5.6%. DNA sequencing showed p53 gene mutation was found in 22 of 36 patients (61.1% ), and p53 gene mutation combined with the methylation of tumor suppressor was found in 14 (38.9%) patients, which was significantly correlated with pathologic biology, invasion, and differentiation ( P ＜ 0.05 ). The 1-year, 2-year, and 3-year survival rates were significantly higher in tumor-suppressing genes methylation group ( n = 4) (70%, 43 %, and 28%, respectively)than those in p53 gene mutation group (n = 14) (28%, 5%, and 0%, respectively) (χ2 =9. 060, P =0.03).Conclusions Promoter hypermethylation of p53-Bax mitochondrial apoptosis pathway is a common epigenetic event in cholangiocarcinoma. Although the methylations of TMS1/ASC and DAPK genes in the adjacent tissues are relatively low, they may be informative for the early detection of cholangiocarcinoma. P53 gene mutation combined with the methylation of tumor suppressor may be related with the pathologic biology of cholangiocarcinoma, making the latter trend to be with high malignancy and poor prognosis.

Objective: To investigate the safety and efficacy of transcatheter perforation of pulmonary valve by micro-guidewire and balloon dilation in the treatment of neonatal pulmonary atresia with intact ventricular septum (PA-IVS). Methods: The retrospective study included 21 neonates (14 males and 7 females) with PA-IVS who underwent transcatheter micro-guidewire pulmonary valve perforation and balloon dilation in Xinhua Hospital from January 2012 to December 2018. All patients underwent the pulmonary valve perforation by micro-guidewire through the Simmons catheter. During the follow-up period at 1, 3, 6, 12 months postoperatively and annually thereafter, the operative efficacy and the development of the right ventricle (RV) were evaluated by echocardiography. Statistical analyses were performed using t test. Results: A total of 21 neonates with PA-IVS were enrolled, and 13 cases were diagnosed prenatally. The median age of surgery was 6 days, the weight was (3.2±0.5) kg. The balloon/valve ratio was 1.19±0.12, and the RV pressure measured by catheter was (121±33) mmHg (1 mmHg=0.133 kPa) . The immediate postoperative RV pressure was (47±13) mmHg. The median follow-up time was 30 months. All the cases enrolled achieved biventricular circulation without death and serious complications. According to the last follow-up data including 16 cases who were followed up for 1 year or longer, the pulmonary artery transvalvular pressure was (29±15) mmHg. The postoperation ratio of right to left ventricular transverse diameter was significantly higher than that before operation (0.86+0.10 vs. 0.73+0.13, t=-2.96, P=0.006). Compared with preoperative data, the postoperation pulmonary valvular diameter Z-score was significantly higher (-1.41±0.89 vs. -2.83±1.06, t=-3.65, P=0.001) and the tricuspid valvular diameter Z-score was significantly higher (-0.52±0.29 vs. -1.34±0.81, t=-3.55, P=0.001). Conclusion Transcatheter perforation of pulmonary valve by micro-guidewire and balloon dilation is a safe and effective initial therapy for neonatal PA-IVS.

OBJECTIVES: Glioma is the most common intracranial primary tumor in central nervous system. Glioma grading possesses important guiding significance for the selection of clinical treatment and follow-up plan, and the assessment of prognosis. This study aims to explore the feasibility of logistic regression model based on radiomics to predict glioma grading. METHODS: Retrospective analysis was performed on 146 glioma patients with confirmed pathological diagnosis from January, 2012 to December, 2018. A total of 41 radiomics features were extracted from contrast-enhanced T RESULTS: A total of 5 imaging features selected by LASSO were used to establish a logistic regression model for predicting glioma grading. The model showed good discrimination with AUC value of 0.919. Hosmer-Lemeshow test showed no significant difference between the calibration curve and the ideal curve ( CONCLUSIONS The logistic regression model using radiomics exhibits a relatively high accuracy for predicting glioma grading, which may serve as a complementary tool for preoperative prediction of giloma grading.
Brain Neoplasms/diagnostic imaging* ; Glioma/diagnostic imaging* ; Humans ; Logistic Models ; Magnetic Resonance Imaging ; ROC Curve ; Retrospective Studies