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Objective: To investigate the incidence, clinical and genetic characteristics of pediatric lymphoma patients of China with inborn errors of immunity (IEI)-related gene mutations, which have not been fully studied. Method: From Jan. 2020 to Mar. 2023, IEI-related genetic mutations were retrospectively explored in 108 children with lymphomas admitted to Beijing Children's Hospital by NGS. Genetic rule and clinical characteristics as well as treatment outcomes were compared between patients with or without IEI-related gene mutations. Results: A total of 17 patients (15.7 %) harbored IEI-associated mutations, including 4 cases with X-linked lymphoproliferative syndrome (XLP), 3 cases had mutations in tumor necrosis factor receptor superfamily 13B (TNFRSF13B), 2 cases with Activated p110 syndrome (APDS). Patients with IEI all had alteration of immunocompetence with decreased levels of immunoglobulin and lymphocyte subsets. Recurrent infection existed in 41.2 % of patients. The 18-month event-free survival (EFS) and the overall response rate (ORR) of patients with IEI are significantly lower than those without IEI (33.86% vs. 73.26 %, p = 0.011; 52.94% vs. 87.91 %, p = 0.002, respectively). In addition, patients with IEI had a higher progression disease (PD) rate of 23.5 % than those without IEI of 4.4 % (p = 0.006). Conclusion: The present study demonstrated that IEI-associated lymphomas were much more common than originally appreciated in pediatric lymphomas, and those were insensitive to treatment and more likely to progress or relapse. The genomic analysis and a thorough review of the medical history of IEI can be used to distinguish them from pediatric lymphomas without IEI, which are beneficial for the early diagnosis and direct intervention. [ABSTRACT FROM AUTHOR]

At the High Energy Photon Source (HEPS; Beijing, People's Republic of China), where up to 90 beamlines can be provided in the future, minimization of workload for individual beamlines and maximization of knowledge about one beamline that can be applied to other beamlines is essential to minimize the total complexity in beamline control. Presented in this paper are our efforts to achieve these goals by composing relatively simple utilities and mechanisms to automate tasks, while always remembering to keep our automation solutions simple and clear. After an introduction to our choice of basic software in EPICS-based beamline control, the issues encountered in introducing package management to EPICS modules, as well as solutions to them, are presented; the design and implementation of the packaging system is concisely discussed. After a presentation of our efforts to reduce the need for self-built multi-device EPICS 'input/output controller' (IOC) applications by providing reusable modular IOC executables, implementation of easily maintainable multi-IOC setups through the separation and minimization of each user's IOC configurations is given. The ongoing project of comprehensive beamline services at HEPS to further simplify configuration management on multiple scales, ranging from individual beamline devices to all beamlines at HEPS, is introduced. [ABSTRACT FROM AUTHOR]

Objective To assess the relationship between fine particulate matter (PM2.5) concentration and ischaemic heart disease (IHD) morbidity and mortality. Methods A time-series study conducted in Beijing from 1 January 2010 to 31 December 2012. Data on 369469 IHD cases and 53247 IHD deaths were collected by the Beijing Monitoring System for Cardiovascular Diseases, which covers all hospital admissions and deaths from IHD from Beijing's population of 19.61 million. Results The mean daily PM2.5 concentration was 96.2 μg/m³ with a range from 3.9 to 493.9 μg/m³. Only 15.3% of the daily PM2.5 concentrations achieved WHO Air Quality Guidelines target (25 μg/m³) in the study period. The dose-response relationships between PM2.5 and IHD morbidity and mortality were non-linear, with a steeper dose-response function at lower concentrations and a shallower response at higher concentrations. A 10 μg/m³ increase in PM2.5 was associated with a 0.27% (95% CI 0.21 to 0.33%, p<2.00×10-16) increase in IHD morbidity and a 0.25% (95% CI 0.10 to 0.40%, p=1.15×10-3) increase in mortality on the same day. During the 3 years, there were 7703 cases and 1475 deaths advanced by PM2.5 pollution over expected rates if daily levels had not exceeded the WHO target. Conclusions PM2.5 concentration was significantly associated with IHD morbidity and mortality in Beijing. Our findings provide a rationale for the urgent need for stringent control of air pollution to reduce PM2.5 concentration. [ABSTRACT FROM AUTHOR]

This study was performed to investigate the prevalence of the metabolic syndrome using a large representative sample in Beijing. Data from a total of 16442 adults (6489 men and 9953 women) aged ≥18 years from a survey of behavioral risk factors for chronic diseases in Beijing, in 2005, was analyzed. The prevalence of the metabolic syndrome increased with age and the age-standardized prevalence of the metabolic syndrome defined by International Diabetes Federation IDF and National Cholesterol Education Program Adult Treatment Panel III ATPIII criteria were 23.2% (24.5% in men and 22.7% in women) and 16.2% (16.1% in men and 16.6% in women), respectively. The metabolic syndrome was higher in semi-urban areas and associated with higher rates of hypertension, central obesity, salt intake and smoking. [ABSTRACT FROM AUTHOR]

Abstract: Eighty road dust-fall (DF) monitoring sites and 14 background monitoring sites were established in the Beijing metropolitan area, and monitoring was conducted from January 2006 to December 2008. The dust-fall attributable to roads (ΔDF) showed a clear decline from 2006 to 2008. Dust-fall levels decreased across different road types from freeway > major arterial roads > minor arterial roads > collector roads > background sites. The ΔDF showed declines of 65%, 55%, 65% and 84% respectively for freeways, major arterial, minor arterial and collector roads from August 2007 to August 2008, and declines of 77%, 76%, 82% and 82% between August 2006 and August 2008. The ΔDF declined by 80%, 79%, 82% and 69% for freeways, major arterial, minor arterial and collector roads respectively between September 2007 and September 2008, and declined by 84%, 88%, 80% and 81% between September 2006 and September 2008. Eighty samples were collected in August 2007 and August 2008 and analyzed for silt loading. PM10 emission factors and emission strengths were calculated using the AP-42 model. The silt loading reduced by 77%, 35%, 61%, 59% and 75% for freeways, major arterial, minor arterial, collector and local roads respectively. The PM10 emission factors were reduced by 57%, 15%, 36%, 51% and 61% and the PM10 emission strength declined by 70%, 40%, 55%, 65% and 72% for freeways, major arterial, minor arterial, collector and local roads respectively between August 2007 and August 2008. The decline is consistent with the reduction in road dust-fall. [Copyright &y& Elsevier]