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.

Background::Intrauterine valvuloplasty is an innovative therapy, which promotes ventricular growth and function in some congenital heart diseases (CHDs). The technique remains challenging and can only be performed in a few centers. This study aimed to assess the feasibility and mid-term outcomes of fetal cardiac intervention (FCI) in fetuses with critical CHD in an experienced tertiary center.Methods::Five fetal aortic valvuloplasty (FAV) or fetal pulmonary valvuloplasty (FPV) procedures were performed in our fetal heart center between August 2018 and May 2022. Technical success was defined as crossing the aortic or pulmonary valve and balloon inflation, followed by evidence of increased blood flow across the valve and/or new regurgitation. Follow-up clinical records and echocardiography were obtained during the prenatal and postnatal periods.Results::Five fetuses received FAV or FPV, including critical aortic stenosis ( n = 2) and pulmonary atresia with intact ventricular septum ( n = 3). The mean maternal age was 33.0 ± 2.6 years. The median gestational age (GA) at diagnosis was 24 weeks (range, 22-26 weeks). The median GA at intervention was 29 weeks (range, 28-32 weeks). All five cases underwent successful or partially successful procedures. One patient had pulmonary valve perforation without balloon dilation. No procedure-related deaths or significant complications occurred. However, one neonatal death occurred due to heart and renal failure. The median follow-up period was 29.5 months (range, 8.0-48.0 months). The four surviving patients had achieved biventricular circulation, exhibited improved valve, and ventricular development at the last follow-up visit. Conclusion::Intrauterine FCI could be performed safely with good prognosis in critical CHD.

PURPOSE: Hrd1 has recently emerged as a critical regulator of B-cells in autoimmune diseases. However, its role in the pathogenesis of chronic rhinosinusitis with nasal polyps (CRSwNP) remains largely unexplored. This study aimed to examine Hrd1 expression and B-cell accumulation and their possible roles in CRSwNP. METHODS: Quantitative real-time polymerase chain reaction, immunohistochemistry, enzyme-linked immunosorbent assay and Western blotting were used to assess gene and protein expression in nasal tissue extracts. Cells isolated from nasal tissues and peripheral blood mononuclear cells were characterized by flow cytometry. Local antibody production was measured in tissue extracts with a Bio-Plex assay. Additionally, changes in Hrd1 expression in response to specific inflammatory stimuli were measured in cultured dispersed polyp cells. RESULTS: Nasal polyps (NPs) from patients with eosinophilic CRSwNP (ECRS) had increased levels of Hrd1, B-cells and plasma cells compared with NPs from patients with non-eosinophilic CRSwNP (non-ECRS) or other control subjects (P < 0.05). The average Hrd1 levels in B-cells in NPs from ECRS patients were significantly higher than those from non-ECRS patients and control subjects (P < 0.05). NPs also contained significantly increased levels of several antibody isotypes compared with normal controls (P < 0.05). Interestingly, Hrd1 expression in cultured polyp cells from ECRS patients, but not non-ECRS patients, was significantly increased by interleukin-1β, lipopolysaccharide and Poly(I:C) stimulation, and inhibited by dexamethasone treatment (P < 0.05). CONCLUSIONS: Differential Hrd1 expression and B-cell accumulation between the ECRS and non-ECRS subsets suggests that they can exhibit distinct pathogenic mechanisms and play important roles in NP.
Antibody Formation ; Autoimmune Diseases ; B-Lymphocytes* ; Blotting, Western ; Dexamethasone ; Enzyme-Linked Immunosorbent Assay ; Eosinophils* ; Flow Cytometry ; Humans ; Immunity, Innate ; Immunohistochemistry ; Nasal Polyps* ; Plasma Cells ; Polyps ; Real-Time Polymerase Chain Reaction ; Tissue Extracts

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.