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Not all stents are suitable for children. For instance, premounted stents can be used in infants and small children but cannot dilate with age to accommodate adult-sized pulmonary arteries. Conversely, the Pul-Stent adapts to somatic growth. Thus, our hospital implemented the Pul-Stent in pediatric patients with branch pulmonary artery stenosis. This study summarizes our initial experience with Pul-Stents in this patient population, including the efficacy and safety. We implanted 37 Pul-Stents in 35 patients between August 2014 and June 2015. The patients’ mean age and weight at stent implantation were 6.7 ± 3.0 years and 20.9 ± 8.7 kg, respectively. Bench testing revealed that axial shortening of the Pul-Stent was minimal with further dilation, and the radial strength did not change. The stents were successfully deployed in all cases, except two with minor malpositioning. Primarily, 8–12 mm mounting balloons were used for the initial implantation, and a long sheath (8–10 F) was used for delivery. After stent implantation, the minimal lumen diameter in the stenosed segment increased by 50% in 97% (34/35) of patients. Furthermore, the pressure gradient across the stenosed segment decreased by 50% in 77% (23/30) of biventricular patients. One stent fracture and one stent restenosis were noted during the follow-up visits (mean follow-up time: 4.6 ± 1.7 years). Eighteen patients (51%) underwent repeat catheterization; ten had successful redilation. No aneurysms or stent fractures were observed. Our initial results indicate that the Pul-Stent is safe and effective in pediatric patients and can be further dilated over time to accommodate somatic growth. Moreover, the Pul-Stent has good compliance and adequate radial strength to treat pulmonary artery stenosis effectively.

Background We aimed to assess the efficacy of different initial intravenous immunoglobulin (IVIG) regimens in Kawasaki disease (KD) patients to find more cost‐effective therapy options. Methods A multicenter, open‐label, blind‐endpoint randomized controlled trial was conducted from January 2014 to December 2015. Patients with KD, within 10 days of illness, were randomly assigned to receive different IVIG regimens (Group A, 2 g/kg once; Group B, 1 g/kg for 2 consecutive days; Group C, 1 g/kg once) and aspirin 30mg/kg/d. Primary outcomes included hours to defervescence and development of coronary artery lesions during the study period. Major secondary outcomes included total fever days, total dose of IVIG, changes of laboratory data, length of stay, and hospitalization expenses. (ClinicalTrials.gov: NCT02439996). Results A total of 404 patients underwent randomization. No difference was found in the outcomes of defervescence among three groups at 6, 12, 24, and 36 hours after completion of initial IVIG infusion. There were no differences in the incidence of coronary artery lesions during the study period (at week 2, month 1, month 3, and month 6 of illness), changes of laboratory data, total fever days, and length of stay. Group C patients had the lowest total dose of IVIG (mean: 1.2 vs 2.2 vs 2.1 g/kg; P < 0.001) and hospitalization expenses (mean: 8443.8 vs 10798.4 vs 11011.4 Chinese Yuan; P < 0.001) than other two groups. Conclusions A single dose of 1g/kg IVIG is a low‐cost treatment with the same efficacy as 2 g/kg IVIG and can be an option for the initial therapy of KD patients.

Increasing evidence has highlighted the endogenous production of formaldehyde (FA) in a variety of fundamental biological processes and its involvement in many disease conditions ranging from cancer to neurodegeneration. To examine the physiological and pathological relevance and functions of FA, fluorescent probes for FA imaging in live biological samples are of great significance. Herein we report a systematic investigation of 2-aza-Cope reactions between homoallylamines and FA for identification of a highly efficient 2-aza-Cope reaction moiety and development of fluorescent probes for imaging FA in living systems. By screening a set of N-substituted homoallylamines and comparing them to previously reported homoallylamine structures for reaction with FA, we found that N-p-methoxybenzyl homoallylamine exhibited an optimal 2-aza-Cope reactivity to FA. Theoretical calculations were then performed to demonstrate that the N-substituent on homoallylamine greatly affects the condensation with FA, which is more likely the rate-determining step. Moreover, the newly identified optimal N-p-methoxybenzyl homoallylamine moiety with a self-immolative β-elimination linker was generally utilized to construct a series of fluorescent probes with varying excitation/emission wavelengths for sensitive and selective detection of FA in aqueous solutions and live cells. Among these probes, the near-infrared probe FFP706 has been well demonstrated to enable direct fluorescence visualization of steady-state endogenous FA in live mouse brain tissues and elevated FA levels in a mouse model of breast cancer. This study provides the optimal aza-Cope reaction moiety for FA probe development and new chemical tools for fluorescence imaging and biological investigation of FA in living systems.

Bilayer graphene (BLG) shows great application prospect and potential in next-generation electronics because of its unique electrical and mechanical properties. However, the scalable synthesis of large-area high-quality BLG films is still a great challenge, despite the maturity of chemical vapor deposition (CVD) technique. In this study, we report a robust method to grow BLGs on flat, softened Cu foils by atmospheric pressure CVD. A moderate amount of residual oxygen accelerates the growth of BLG domains while suppressing the formation of multilayers. Raising the nucleation density at low hydrogen pressure efficiently increases the film continuity. Based on the optimized CVD process, the growth of graphene films on 4×4 cm2 Cu foils with an average BLG coverage of 76% is achieved. The morphology and structure characterizations demonstrate a high quality of the BLG. Dual gate field-effect transistors are investigated based on AB-stacked BLG, with a tunable bandgap and high carrier mobility of up to 6790 cm2 V−1 s−1 at room temperature.

More than 50% of Chinese college students rarely engage in any type of physical activity (PA). The literature shows several issues in college students’ health due to lack of PA. Promoting college students’ PA participation across the country has become a challenging task. This study aims to create a questionnaire into the correlates that affect Chinese college students’ engagement in leisure time PA. We first determined the factor structure of college students’ PA engagements. Then, we tested and verified the reliability and validity of the factor structure of the questionnaire.TheChinese college students’ PA correlates questionnaire was designed according to the Social Ecological Model. Exploratory factor analysis (EFA) extracted all the included items, whereas confirmatory factor analysis (CFA) verified the validity of the model. We recruited 1290 college students to complete the questionnaire. A second-order structural model was constructed. Specifically, the first-order included individual, social, and environmental as dimensions, while the second-order had interest, media, social support, traditional culture, facilities, and environment as factors. The six factors have polymerized 22 items. The model demonstrated a good fit (X2/df = 1.4; CFI = 0.98; GFI = 0.94; RMSEA = 0.02; SRMR = 0.05). In conclusion, the newly established questionnaire of college students’ PA correlates is reasonable, and the results of factors reliabilities and model fit are acceptable.

The straightforward strategy of building a chiral C-O bond directly on a general carbon radical center is challenging and stereocontrol of the reactions of open-chain hydrocarbon radicals remains a largely unsolved problem. Advance in this elementary step will spur the development of asymmetric radical C-O bond construction. Herein, we report a copper-catalyzed regioselective and enantioselective carboesterification of substituted dienes using alkyl diacyl peroxides as the source of both the carbon and oxygen substituents. The participation of external acids in this reaction substantially extends its applicability and leads to structurally diverse allylic ester products. This work represents the advance in the key elementary reaction of intermolecular enantioselective construction of C-O bond on open-chain hydrocarbon radicals and may lead to the discovery of other asymmetric radical reactions., Stereocontrol of C–O bond formation from a carbon-based radical is very difficult due to the rapid inversion of the carbon radical. Here the authors present a method to form chiral esters from conjugated dienes with copper and chiral PyBox ligands, likely proceeding via an allylic radical.

Cardiovascular disease is a leading cause of death and disability worldwide. Although genetically modified mouse models offer great potential for robust research in vivo, in vitro studies using isolated cardiomyocytes also provide an important approach for investigating the mechanisms underlying cardiovascular disease pathogenesis and drug actions. Currently, isolation of mouse adult cardiomyocytes often relies on aortic retrograde intubation under a stereoscopic microscope, which poses considerable technical barriers and requires extensive training. Although a simplified, Langendorff‐free method has been used to isolate viable cardiomyocytes from the adult mouse heart, the system requires enzymatic digestions and continuous manual technical operation. This study established an optimized approach that allows isolation of adult mouse cardiomyocytes and epicardial activation mapping of mouse hearts using a Langendorff device. We used retrograde puncture through the abdominal aorta in vivo and enzymatic digestion on the Langendorff perfusion device to isolate adult mouse cardiomyocytes without using a microscope. The yields of isolated cardiomyocytes were amenable to patch clamp techniques. Furthermore, this approach allowed epicardial activation mapping. We used a novel, simplified method to isolate viable cardiomyocytes from adult mouse hearts and to map epicardial activation. This novel approach could be beneficial in more extensive research in the cardiac field.

Increasing evidence has highlighted the endogenous production of formaldehyde (FA) in a variety of fundamental biological processes and its involvement in many disease conditions ranging from cancer to neurodegeneration. To examine the physiological and pathological relevance and functions of FA, fluorescent probes for FA imaging in live biological samples are of great significance. Herein we report a systematic investigation of 2-aza-Cope reactions between homoallylamines and FA for identification of a highly efficient 2-aza-Cope reaction moiety and development of fluorescent probes for imaging FA in living systems. By screening a set of N-substituted homoallylamines and comparing them to previously reported homoallylamine structures for reaction with FA, we found that N-p-methoxybenzyl homoallylamine exhibited an optimal 2-aza-Cope reactivity to FA. Theoretical calculations were then performed to demonstrate that the N-substituent on homoallylamine greatly affects the condensation with FA, which is more likely the rate-determining step. Moreover, the newly identified optimal N-p-methoxybenzyl homoallylamine moiety with a self-immolative β-elimination linker was generally utilized to construct a series of fluorescent probes with varying excitation/emission wavelengths for sensitive and selective detection of FA in aqueous solutions and live cells. Among these probes, the near-infrared probe FFP706 has been well demonstrated to enable direct fluorescence visualization of steady-state endogenous FA in live mouse brain tissues and elevated FA levels in a mouse model of breast cancer. This study provides the optimal aza-Cope reaction moiety for FA probe development and new chemical tools for fluorescence imaging and biological investigation of FA in living systems., Systematic investigation of various homoallylamines reveals N-p-methoxybenzyl homoallylamine as the optimal 2-aza-Cope reaction moiety for development of highly efficient formaldehyde fluorescent probes for in vitro and in vivo imaging.

Summary Polarity-reversal catalysts enable otherwise sluggish or completely ineffective reactions which are characterized by unfavorable polar effects between radicals and substrates. We herein disclose that when irradiated by visible light, bromine can behave as a polarity-reversal catalyst. Hydroacylation of vinyl arenes, a three-component cascade transformation and deuteration of aldehydes were each achieved in a metal-free manner without initiators by using inexpensive N-bromosuccinimide as the precatalyst. Light is essential to generate and maintain the active bromine radical during the reaction process. Another key to success is that HBr can behave as an effective hydrogen donor to turn over the catalytic cycles., Graphical abstract, Highlights • Using bromine as a polarity-reversal catalyst to generate acyl radicals • Additive- and metal-free, atom- and step-economic, and operationally simple process • Using constant light-irradiation to induce and maintain bromine radicals • Access carbonyl compounds and deuterated aldehydes with wide substrate scope, Chemistry; Organic chemistry; Green chemistry

Narrowing the bandgap of lead-free double-perovskite Cs2AgBiBr6 is required for using this material in future photovoltaics. Herein, we demonstrate a bandgap engineering of Cs2AgBiBr6 by introducing Sb to substitute up to 75% of Bi via a versatile solution-processed method in dimethyl sulfoxide at 180 °C. The resultant Cs2AgSbxBi1−xBr6 (x = 0, 0.25, 0.50, 0.75) thin films possess high crystallinity and good thermostability. Moreover, the Sb substitution enables an obvious bandgap reduction of 0.25 eV. The fabricated solar cell using the Cs2AgSbxBi1−xBr6 (x = 0.25) thin film obtained an increased performance than the reference Cs2AgBiBr6. The effective bandgap narrowing via a facile solution method might accelerate the development of Cs2AgBiBr6-based materials for photovoltaic applications.

Polarity-reversal catalysts enable otherwise sluggish or completely ineffective reactions which are characterized by unfavorable polar effects between radicals and substrates. We herein disclose for the first time that when irradiated by visible light, bromine can behave as a polarity-reversal catalyst. Hydroacylation of vinyl arenes, a three-component cascade transformation and deuteration of aldehydes were each achieved in a metal-free manner without initiators by using N-bromosuccinimide as the precatalyst. Light is essential to generate and maintain the active bromine radical during the reaction process. Another key to success is that HBr can behave as an effective hydrogen-donor to turn over the catalytic cycles.

Background: Postoperative thrombocytopenia with congenital heart disease (CHD) after percutaneous transcatheter closure (PTC) has seldom been reported. We sought to investigate the treatments, severity and causes of postoperative thrombocytopenia among patients with CHD undergoing PTC.Methods: Six PTC patients undergoing postoperative thrombocytopenia in our department were included. Platelet count was measured at admission and daily after PTC at least for day 9.Results: Three patients had platelet counts below 30×10^9 /L while in the other two patients the counts were below 50×10^9 /L. All patients achieved a stable platelet count, ranging from 99×10^9 /L to 241×10^9 /L after day 9. The platelet counts recovered after being given intravenously the methylprednisolone (two patients), immunoglobulin (one patient) and platelet transfusions (two patients). During the whole course, the face and limbs of one patients were visible ecchymosis but no major bleeding events were recorded.Conclusion: In this report, the clinical outcome of our patients were satisfactory; they complaints regressed after above treatment.

Autotransporters are important virulence factors in the outer membrane of gram-negative bacteria. Although several autotransporters have been identified in Neisseria meningitidis, only IgA1 protease has been identified in Neisseria gonorrhoeae. A sequence analysis showed a marked difference in the distribution of autotransporters between the two strains. It has been speculated that only two autotransporters, the IgA1 protease and the NGO2105 protein, might be encoded by N. gonorrhoeae. Here, we describe the identification of NGO2105, a new autotransporter in N. gonorrhoeae. A sequence alignment showed that NGO2105 is highly similar to the adhesion and penetration protein (App) in N. meningitidis. We found that NGO2105 is exported to the outer membrane, cleaved and released into the culture supernatant by endogenous serine protease activity in N. gonorrhoeae and E. coli. The site-directed mutagenesis of S267A in the predicted enzyme catalytic triad abolished autoproteolytic cleavage to allow secretion. The NGO2105 β-barrel shows the ability to translocate the heterologous Hbp passenger domain. NGO2105 is involved in gonococcal adherence to and invasion into human cervical epithelial cells. Furthermore, antibodies raised against NGO2105 are able to block gonococcal adherence to human cervical epithelial cells. The Δngo2105 mutant and anti-NGO2105 antiserum significantly attenuated the colonization of N. gonorrhoeae in mice. Collectively, our results suggest that the newly identified serine protease autotransporter NGO2105 represents a novel virulence factor of gonococcus and a potential vaccine target.

“PlantNanOmics” is an emerging topic in agricultural research that explores the potential effect of application of nanomaterials on plant growth. Graphene oxide (GO) has excellent properties due to its basal carbon plane and oxygen-containing functional groups. In the present work, the antimicrobial activity of GO was exploited to extend the vase life and improve the quality of cut roses (cv. Carola). The results revealed that the cut roses cultivated in low doses of GO (0.1 mg/L) had longer vase life, larger diameter, and better water relations. Microbial contaminations at the basal stem end is the most common reason for stem blockage that causes water stress and early wilting of cut flowers. GO was found to act as a germicide, effectively inhibiting the microbial growth at the cut stem end and improving water uptake and water balance of cut roses. Therefore, GO can serve as a promising preservative to increase the ornamental value of cut flowers.

Background DegS is a periplasmic serine protease that is considered to be the initiator of the σE stress response pathway, and this protein plays an important role in the regulation of the stress response in E. coli. However, knowledge of the biological function and global regulatory network of DegS in Vibrio cholerae remains limited. In this study, we aimed to characterize the molecular functions and further investigate the regulatory network of degS in V. cholerae. Methods A deletion mutant of degS was constructed in the V. cholerae HN375 strain. Bacterial colony morphology was observed by a plate-based growth experiment, and bacterial growth ability was observed by a growth curve experiment. High-throughput RNA sequencing (RNA-Seq) technology was used to analyze the differential transcriptomic profiles between the wild-type and degS mutant strains. Gene ontology (GO), pathway analysis and Gene-Act-network analysis were performed to explore the main functions of the differentially expressed genes. Quantitative real-time PCR (qRT-PCR) was performed to validate the reliability and accuracy of the RNA-Seq analysis. The complementation experiments were used to test the roles of degS and ropS in the small colony degS mutant phenotype. Results When degS was deleted, the degS mutant exhibited smaller colonies on various media and slower growth than the wild-type strain. A total of 423 differentially expressed genes were identified, including 187 genes that were upregulated in the degS mutant compared to the wild-type strain and 236 genes that were relatively downregulated. GO categories and pathway analysis showed that many differentially expressed genes were associated with various cellular metabolic pathways and the cell cycle. Furthermore, Gene-Act network analysis showed that many differentially expressed genes were involved in cellular metabolic pathways and bacterial chemotaxis. The cAMP-CRP-RpoS signaling pathway and the LuxPQ signal transduction system were also affected by the degS mutant. The expression patterns of nine randomly selected differentially expressed genes were consistent between the qRT-PCR and RNA-seq results. The complementation experiments showed that the small colony degS mutant phenotype could be partially restored by complementation with the pBAD24-degS or pBAD24-rpoS plasmid. Discussion These results suggest that the degS gene is important for normal growth of V. cholerae. Some of the differentially expressed genes were involved in various cellular metabolic processes and the cell cycle, which may be associated with bacterial growth. Several new degS-related regulatory networks were identified. In addition, our results suggested that the cAMP-CRP-RpoS signaling pathway may be involved in the small colony degS mutant phenotype. Overall, we believe that these transcriptomic data will serve as useful genetic resources for research on the functions of degS in V. cholerae.

Background Open cardiac surgery has traditionally been the gold standard for repair of ventricular septal defect (VSD). The inherent risks and complications associated with open surgery and the incidence of postoperative residual VSD are significant disadvantages of the open surgical approach. Objective To evaluate the methodology and efficacy of transcatheter closure of postoperative residual VSD in children. Methods Patients with postoperative VSD who underwent percutaneous device closure in the period from August 2010 and February 2014 in our center were analyzed retrospectively. Twelve children, including 8 males and 4 females, with mean age of 8.13 ± 5.39 years (range 0.67–17 years) and mean weight of 29.63 ± 20.86 kg (range 5.5–66 kg) were included in the study. Results Ten cases had residual shunts situated at the margin of the patches while the other two had residual shunts because of multiple muscular VSD. The mean VSD diameter was 3.84 ± 1.86 mm (range 2.3–8.7 mm). On cardiac catheterization, the reported mean pulmonary circulatory blood volume/systemic circulation volume (Qp/Qs) ratio was 1.86 ± 0.91 (range 1.1–4.25) and the mean pulmonary artery pressure was 25.92 ± 12.42 mm Hg (range 7–52 mm Hg). Successful transcatheter closure was obtained in all twelve patients with postsurgery residual VSD. Two cases with residual muscular VSD underwent retrograde catheterization via the femoral artery while one case with residual VSD located at the lower margin of the patch was accessed via the internal jugular vein. The mean procedure time was 81.25 ± 25.86 minutes (range 40–120 minutes). There was no evidence of residual VSD, and no instances of new-onset valvular regurgitation were reported, with the only two exceptions being patients who had multiple muscular VSDs where clinically insignificant residual shunts were detected. Conclusion Our retrospective study highlights the potential safety and therapeutic efficacy of transcatheter approach for closing postoperative residual VSD in children. Recommendations More robust studies with longer-term follow-up of outcomes are required to firmly establish the safety profile and respective indications for use of various occluder devices for treating different categories of residual VSD.

In recent decades, increasing numbers of human infections have been linked to non-O1/non-O139 Vibrio cholerae. Septicemia resulting from non-O1/non-O139 V. cholerae infection is rare but has high mortality. The pathogenesis of non-O1/non-O139 V. cholerae septicemia is poorly understood. Here, we report two sporadic cases of septicemia following non-O1/non-O139 V. cholerae infection from an inland area of China. Patient 1 died rapidly within 24 hours, while patient 2 gradually recovered from septic shock. To explore the reasons for these divergent outcomes, we compared the two cases, tested the antibiotic sensitivity of the two isolates, and investigated their virulence genes and sequence types.

Teaching reform is a new curriculum resource based on the Internet and information technology (IT). In higher vocational English teaching, a new model of “Teaching after learning, teaching based on learning” has been implemented in teaching reform. In particular, supported by the mobile Moodle platform, the reform of higher vocational English teaching has played a positive role in facilitating the improvement of listening, speaking, reading, writing, and translation levels in students. In this paper, based on the current higher vocational English teaching practice, the development of English teaching reform supported by the mobile Moodle platform is explored. In addition, the contents of English speaking, reading, writing, translation, etc. are combined to discuss how to use the Moodle teaching reform to improve the English teaching effectiveness.

Background The RASopathies are a class of developmental disorders caused by germline mutations in the RAS-mitogen-activated protein kinase (MAPK) pathway. Hypertrophic cardiomyopathy (HCM) has been frequently described in children with RASopathy, but only a minority of patients have received formal genotyping. The purpose of this study was to evaluate the genetic basis and clinical outcome of pediatric patients with RASopathy-associated HCM. Methods We retrospectively reviewed the mutation spectrum and clinical outcome of all the patients with RASopathy derived from 168 pediatric HCM cases referred to our institution between January 2012 and July 2018. Results A heterozygous missense mutation in one of known RASopathy genes was identified in 46 unrelated children with HCM. Mutations in the PTPN11 gene were the most prevalent (19/46); this was followed by mutations in RAF1 (11/46), KRAS (5/46), RIT1 (4/46), BRAF (3/46), SOS1 (2/46), HRAS (1/46), and SHOC2 (1/46). Moreover, two compound heterozygous missense mutations in the LZTR1 gene were identified in one patient with the Noonan syndrome phenotype and HCM. The median age at the diagnosis of HCM was 3.0 months (range 0 months to 8.1 years). Twenty-one of the patients had significant left ventricular outflow tract obstruction and 32 had concomitant congenital heart disease. Three patients with a mutation in exon 13 of the PTPN11 gene died of cardiac failure at the ages of 3.0, 3.5, and 6.0 months. The remaining 44 patients were alive after an average follow-up time of 3.9 years (0.5 to 17.1 years, median 2.9 years) from the initial diagnosis of HCM, including 5 patients with spontaneous regression of their cardiac hypertrophy. Conclusions RASopathy-associated HCM is a heterogeneous genetic condition characterized by early-onset cardiac hypertrophy and a high prevalence of co-existing congenital heart disease, which is most frequently related to specific mutations in the PTPN11 gene. Rapidly progressive HCM, resulting in an early death, is uncommon in RASopathy patients except those with specific mutations in exon 13 of the PTPN11 gene.

Pompe disease is an autosomal recessive disorder and is caused by a deficiency in acid alpha-glucosidase (GAA). A broad range of studies have been performed on Pompe patients from different countries. However, the clinical course and molecular basis of the disease in Mainland China have not been well defined. In the present study, we examined a total of 18 Chinese children with infantile-onset Pompe disease to better understand the clinical and genetic features in this population. The median age at symptom onset was 3.6 months (range: 1.7–6.8 months) and 6.3 months at diagnosis (range: 2.5–9.3 months). All but 1 patient died at a median age of 8.2 months (range: 4.7–18.7 months). Molecular analysis revealed 20 different mutations, 6 of which are novel (c.1356delC, c.378G > A, c.1827C > G, c.859-2 A > T, c.1551 + 2T > G, and c.1465G > T). The most common mutation in the study was c.1935C > A, accounting for 25% (9/36 alleles) of the mutations. Our study provides the first comprehensive examination of the clinical course of infantile-onset Pompe disease and mutations of the GAA gene for patients in Mainland China. Our results confirm the high prevalence of the c.1935C > A mutation, previously reported for other populations, in Mainland Chinese patients with infantile-onset Pompe disease. Furthermore, six novel mutations in the GAA gene are reported for the first time.

Background Barth syndrome (BTHS) is a rare X-linked recessive disease characterized by cardiomyopathy, neutropenia, skeletal myopathy and growth delay. Early diagnosis and appropriate treatment may improve the prognosis of this disease. The purpose of this study is to determine the role of targeted next-generation sequencing (NGS) in the early diagnosis of BTHS in children with cardiomyopathy. Methods During the period between 2012 and 2015, a gene panel-based NGS approach was used to search for potentially disease-causing genetic variants in all patients referred to our institution with a clinical diagnosis of primary cardiomyopathy. NGS was performed using the Illumina sequencing system. Results A total of 180 Chinese pediatric patients (114 males and 66 females) diagnosed with primary cardiomyopathy were enrolled in this study. TAZ mutations were identified in four of the male index patients, including two novel mutations (c.527A > G, p.H176R and c.134_136delinsCC, p.H45PfsX38). All four probands and two additional affected male family members were born at full term with a median birth weight of 2350 g (range, 2000–2850 g). The median age at diagnosis of cardiomyopathy was 3.0 months (range, 1.0–20.0 months). The baseline echocardiography revealed prominent dilation and trabeculations of the left ventricle with impaired systolic function in the six patients, four of which fulfilled the diagnostic criteria of left ventricular noncompaction. Other aspects of their clinical presentations included hypotonia (6/6), growth delay (6/6), neutropenia (3/6) and 3-methylglutaconic aciduria (4/5). Five patients died at a median age of 7.5 months (range, 7.0–12.0 months). The cause of death was heart failure associated with infection in three patients and cardiac arrhythmia in two patients. The remaining one patient survived beyond infancy but had fallen into a persistent vegetative state after suffering from cardiac arrest. Conclusions This is the first report of systematic mutation screening of TAZ in a large cohort of pediatric patients with primary cardiomyopathy using the NGS approach. TAZ mutations were found in 4/114 (3.5%) male patients with primary cardiomyopathy. Our findings indicate that the inclusion of TAZ gene testing in cardiomyopathy genetic testing panels may contribute to the early diagnosis of BTHS.

Open cardiac surgery has traditionally been the gold standard for repair of ventricular septal defect (VSD). The inherent risks and complications associated with open surgery and the incidence of postoperative residual VSD are significant disadvantages of the open surgical approach.To evaluate the methodology and efficacy of transcatheter closure of postoperative residual VSD in children.Patients with postoperative VSD who underwent percutaneous device closure in the period from August 2010 and February 2014 in our center were analyzed retrospectively. Twelve children, including 8 males and 4 females, with mean age of 8.13 ± 5.39 years (range 0.67-17 years) and mean weight of 29.63 ± 20.86 kg (range 5.5-66 kg) were included in the study.Ten cases had residual shunts situated at the margin of the patches while the other two had residual shunts because of multiple muscular VSD. The mean VSD diameter was 3.84 ± 1.86 mm (range 2.3-8.7 mm). On cardiac catheterization, the reported mean pulmonary circulatory blood volume/systemic circulation volume (Qp/Qs) ratio was 1.86 ± 0.91 (range 1.1-4.25) and the mean pulmonary artery pressure was 25.92 ± 12.42 mm Hg (range 7-52 mm Hg). Successful transcatheter closure was obtained in all twelve patients with postsurgery residual VSD. Two cases with residual muscular VSD underwent retrograde catheterization via the femoral artery while one case with residual VSD located at the lower margin of the patch was accessed via the internal jugular vein. The mean procedure time was 81.25 ± 25.86 minutes (range 40-120 minutes). There was no evidence of residual VSD, and no instances of new-onset valvular regurgitation were reported, with the only two exceptions being patients who had multiple muscular VSDs where clinically insignificant residual shunts were detected.Our retrospective study highlights the potential safety and therapeutic efficacy of transcatheter approach for closing postoperative residual VSD in children.More robust studies with longer-term follow-up of outcomes are required to firmly establish the safety profile and respective indications for use of various occluder devices for treating different categories of residual VSD.

The Chinese commonly believe that tea helps maintain clear vision. This viewpoint has been recorded in Chinese medical books also. The key bioactive compounds in green tea leaves, (-)-epigallocatechin gallate (EGCG), L-theanine (theanine) and caffeine, were investigated for their abilities to attenuate the injury of retinal ganglion cells (RGC-5) induced by H2O2 and ultraviolet radiation. Theanine and caffeine promoted cell growth while concentrations of EGCG greater than 10μg/ml inhibited cell growth. The nine and caffeine both protected RGC-5 cells from injury as well as enhanced their recovery, while EGCG only protected the cells from injury and did not help them to recover. Tea is a unique drink, which is simultaneously enriched with EGCG, theanine and caffeine. The role of these compounds in optic nerve protection may partially explain why some tea drinkers feel enhanced vision.

To investigate the role of spd1672 gene in the infection process of Streptococcus pneumoniae.BALB/c mice were intraperitoneally infected by a spd1672 knockout strain and a D39 wild-type strain of S. pneumoniae, and the survival time of mice and blood bacterial counts were recorded. The adhesion and invasion ability of S. pneumoniae strains were assessed in A549 cells. Bactericidal assays were carried out to determine the resistance of spd1672 knockout strains and D39 wild strains, and the serum levels of inflammatory cytokines were detected in the infected mice.The mice infected with spd1672 knockout strains showed a significantly longer median survival time, a higher survival rate, and a lower blood bacterial load than the wild strain-infected mice (P0.05). Having a similar cell adhesion ability to the wild-type strain (P0.05), the spd1672 knockout strain showed significantly lower cell invasion ability than the wild-type strain (P0.05). The spd1672 knockout strain also had a reduced resistance to whole blood cells, and thw mice infected with spd1672 knockout strain exhibit lower levels of serum inflammatory cytokines than those infected with the wild-type strain.Spd1672 gene is importantly related to the virulence of S. pneumoniae and plays important roles in modulating bacterial invasion, resistance to whole blood cells and proinflammatory responses.

Correction for ‘The physics and chemistry of graphene-on-surfaces’ by Guoke Zhao, Xinming Li, Meirong Huang et al., Chem. Soc. Rev., 2017, 46, 4417–4449.