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Background: As the first gene therapy for spinal muscular atrophy (SMA), nusinersen is supposed to be administrated via intrathecal injection regularly for a lifetime. However, for SMA patients with severe spinal deformities, bony fusion following posterior spinal instrumentation sets great obstacles for the application of nusinersen. Therefore, efforts have been devoted to the exploration of appropriate approach for nusinersen administration. This study aims to evaluate the safety and reliability of unilateral interlaminar fenestration on the convex side during spinal fusion surgery for intrathecal nusinersen injection in SMA. Results: SMA patients receiving posterior spinal fusion and interlaminar fenestration in Peking Union Medical College Hospital from January 2020 to October 2021 were retrospectively analyzed. 13 patients were included. Of the 13 patients, 10 were classified into SMA type II and 3 into SMA type III. Distal fusion to pelvis was undertaken in 11 patients; while L5 was selected as the lowest instrumented vertebra in the other 2 patients. All patients received interlaminar fenestration on the convex side only with an area of about 15 mm × 20 mm. Fenestration at L2–L3 level was performed in 6 patients; while L3–L4 level was selected for windowing in the remaining 7 patients. 9 of the 13 patients received lumbar puncture and intrathecal nusinersen administration during the 1-year follow-up, with an accumulative total of 50 times. All injections were performed successfully under ultrasound guidance, with no one transferred to radiographic assistance. No severe complications occurred after injection. Conclusions: In SMA with severe scoliosis planning to receive posterior spinal fusion, unilateral lumbar interlaminar fenestration on the convex side provides a feasible and reliable access for intrathecal nusinersen administration after surgery. [ABSTRACT FROM AUTHOR]

China's public diplomacy has experienced profound changes since the new leadership came to power. The outdated diplomatic principle of 'hide our capacities and bide our time' was gradually replaced by an emerging narrative about 'China's ideas' and 'China's wisdom'. China's economic and social development is reflected in its unique governance model. In theory, good governance is expected to enhance soft power overseas, but China's growing influence is controversially mediated as sharp power. This study scrutinises if and how Beijing is promoting its political ideas as the key pillar of its soft power portfolio and how Australia mediated China's intended narrative. A comparative framing analysis of China's media and in-depth interviews with 18 Australian experts outlines Beijing's ambitions and the dilemma of China's soft power. This research sheds new light on the interplay between domestic governance and soft power by examining the congruity between China's projection and Australia's interpretation. [ABSTRACT FROM AUTHOR]

Objectives: Whole exome sequencing (WES) has been widely used to detect genetic disorders in critically ill children. Relevant data are lacking in pediatric intensive care units (PICUs) of China. This study aimed to investigate the spectrum of monogenic disorders, the diagnostic yield and clinical utility of WES from a PICU in a large children's hospital of China. Methods: From July 2017 to February 2020, WES was performed in 169 critically ill children with suspected monogenic diseases in the PICU of Beijing Children's Hospital. The clinical features, human phenotype ontology (HPO) terms, and assessment of clinical impact were analyzed. Results: The media age of the enrolled children was 10.5 months (range, 1 month to 14.8 years). After WES, a total of 43 patients (25%) were diagnosed with monogenic disorders. The most common categories of diseases were metabolic disease (33%), neuromuscular disease (19%), and multiple deformities (14%). The diagnosis yield of children with "metabolism/homeostasis disorder" and "growth delay" or "ocular anomalies" was higher than that of children without these features. In addition, the diagnosis rate increased when more features were observed in children. The results of WES had an impact on the treatment for 30 cases (70%): (1) change of treatment (n = 11), (2) disease monitoring initiation (n = 18), (3) other systemic evaluation (n = 3), (4) family intervention (n = 2), and (5) rehabilitation and redirection of care toward palliative care (n = 12). Conclusion: WES can be used as an effective diagnostic tool in the PICU of China and has an important impact on the treatment of patients with suspected monogenic conditions. [ABSTRACT FROM AUTHOR]

This study aimed to translate into Chinese the Diabetes Care Profile (DCP), a measure of psychosocial factors and diabetes treatment, and to test the reliability and validity of the instrument within a Chinese population. The English version of the DCP was translated into Chinese following the standard translation methodology with consideration to cultural adaptation. The questionnaire was administered to 313 people with type 2 diabetes in an urban community in Beijing, China. Cronbach’s α coefficient was used to calculate reliabilities, which ranged from .55 to .86 on DCP subscales. Mean values on the DCP differed by diabetes treatment as expected and supports the construct validity of the DCP. The overall score on the DCP correlated well both with blood glucose levels and with a validated measure of a Chinese version of the Diabetes Specific Quality of Life scale, thus supporting the DCP’s criterion validity. The DCP is an acceptable measure of the psychosocial factors related to diabetes and its treatment in a Chinese population with type 2 diabetes. [ABSTRACT FROM PUBLISHER]

We investigated three locations in Beijing, China, containing different industrial plants that may cause pollution of polychlorinated biphenyls (PCBs). The highest soil concentration of 1,000 pg g (dry wt) was found in the chemical plant. The concentrations of ΣPCBs tended to decrease with distance from each of the investigated sites. The principal component analysis demonstrated that there were not substantial differences in PCB homologue patterns among these industrial sites. Tri-CBs and tetra-CBs were the dominant congeners. Based on the data obtained in this investigation, further study of the emission of PCBs from these industrial sites in Beijing is warranted. [ABSTRACT FROM AUTHOR]

Areas containing industrial facilities belonging to three different typical industries that may cause pollution by polybrominated diphenyl ethers (PBDEs) in Beijing, China were investigated. Specifically, the areas contained a solid waste incineration plant, a chemical factory, and a heat and power plant. Investigation of the pollution status of PBDEs in the surface soil from areas around these industries revealed the highest concentration of 42 PBDE congeners (118 ng/g, dry mass) at the solid waste incineration plant. In the other two plants, the highest concentrations were both 26 ng/g (dry mass). Among the PBDE homologues, the PBDE contamination at all sites showed similar congener compositions, with BDE 209 being the dominant congener. Our findings established the first contamination status of three typical industrial areas in Beijing. Furthermore, the total concentrations of 42 PBDE congeners tended to decrease as the distance from the investigated plants increased. Overall, these plants were identified as potential pollution sources of PBDEs in Beijing. Moreover, Pearson correlation analysis revealed that the major PBDE source in Beijing may be associated with the technical deca-BDE and penta-BDE mixture. Based on the data obtained in this preliminary investigation, further study of the potential of these sources to emit PBDEs in Beijing is warranted. [ABSTRACT FROM AUTHOR]

In order to study the decay rate of the rail in some section of Beijing metro using the egg fastening system, a cellular rail model based on the spectral element method is established. In combination with Bloch theorem, the decay rate of the rail with the egg fastening system is hereby studied. On this basis, the influence of tuned rail damper (TRD) on the decay rate of the rail is analyzed. At present, the decay rate of the rail with the egg fastening system is relatively low, in the frequency range from 200 Hz to 400 Hz. The influence of the egg fastening system on the decay rate of the rail is mainly reflected in the frequency range below 450 Hz. TRD can greatly increase the decay rate of the rail in the vicinity of its operating frequency. Moreover, TRD damping can increase the decay rate of the rail, which is beneficial in controlling rail vibration. Rail vibration can be better controlled by increasing the weight of TRD when the operating frequency is constant. The installation position of TRD also affects the decay rate of the rail. Appropriate adjustment of this position can more effectively suppress rail vibrations over a wider frequency range. [ABSTRACT FROM AUTHOR]