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关键词： Waardenburg syndrome type 1   Sensorineural hearing loss   Cochlear implantation   Dystopia canthorum   Resource-limited setting   Newborn hearing screening  

摘要： Waardenburg syndrome (WS) accounts for 2–5% of congenital sensorineural hearing loss (SNHL), and type 1 (WS1) often presents with profound deafness amenable to cochlear implantation (CI). In many low-resource settings without newborn hearing screening or genetic testing, diagnosis and intervention are frequently delayed.

摘要： This study proposes a novel green arylation strategy based on covalent organic framework (COF) photocatalysis. By employing a two-dimensional imine-linked COF (COF-TpPa-1) as a high-performance heterogeneous photocatalyst, this system successfully achieves the direct C3-arylation of quinoxalin-2(1H) -ones with hydrazines under visible-light irradiation. The COF catalyst combines broad-spectrum light-harvesting capability, exceptional structural stability, and facile recyclability, which collectively endow this strategy with high reaction efficiency, broad substrate compatibility, scalability for synthesis, and outstanding catalyst reusability. This work provides an innovative and sustainable catalytic platform for constructing privileged C3-arylated quinoxalin-2(1H) -one scaffolds.

关键词： Nuclear-grade   Dissimilar Welds   Inconel   Martensitic Steels   Delta Ferrite patches   Soft Zone   Ni-1  

摘要： The pursuit of efficiency in nuclear power plant components operating at high steam temperatures and pressures has driven the use of advanced alloys like P91 steel and 304 L stainless steel, yet the structural integrity of their dissimilar welds remains challenged by carbon migration and premature failure; prompting this study to investigate the effectiveness of a Ni-1 (∼92% Ni) intermediate buffer layer combined with ERNiCrMo-3 (IN625) filler metal via multi-pass Gas Tungsten Arc Welding to enhance their metallurgical and mechanical performance. The study compared three IN625 buttering applications: as-welded, post-weld heat-treated, and post-weld heat-treated with an intermediate Ni-1 buffer layer. Microstructural analysis, employing optical microscopy and scanning electron microscopy, revealed peninsula features and an unmixed zone at the interface. The fusion zone features included Mo, Nb, and Ti-rich precipitates. The P91 heat-affected zone demonstrated a soft delta ferrite zone. Mechanical testing showed tensile strengths of 674 MPa (as-welded), 653 MPa (post-weld heat-treated), and 633 MPa (post-weld heat-treated with buffering). Notably, the Ni-1 buffer with post-weld heat treatment resulted in a 14% improvement in P91 heat-affected zone impact toughness compared to the as-welded joint. Microhardness profiling revealed the highest hardness in the coarse-grain heat-affected zone and the lowest in the intercritical heat-affected zone. The study concludes that the Ni-1 buffer layer successfully suppresses carbon migration, preventing the formation of detrimental carbon-depleted and enriched zones. By mitigating metallurgical degradation and improving toughness, the Ni-1/IN625 dual-filler approach provides a robust solution for the structural reliability of P91-304 L SS joints in demanding nuclear applications.

关键词： Zeolite   Sodium silicate   Ultrafast synthesis   Metal nanoclusters encapsulation   Propane dehydrogenation  

摘要： The demand for zeolites is expected to grow rapidly due to their expanding applications in catalysis and separation, which drives the need for efficient synthesis routes based on economical raw materials. To overcome the limitations of time-consuming conventional zeolite synthesis, an ultrafast and low-cost route has been developed using industrial-grade sodium silicate as the silica source. This approach enables minute-level synthesis of silicalite-1 zeolite through a combination of acid neutralization and seeding strategies. Notably, carbon dioxide was also demonstrated as an effective neutralizing agent, offering a sustainable alternative. This ultrafast route further enabled the in-situ encapsulation of metal nanoclusters during zeolite crystallization, which resulted in well-defined metal-zeolite catalysts. Using Pt–Zn as a model system, the catalysts exhibited high activity and long-term stability in propane dehydrogenation. This work presents a scalable and economical route for producing high-performance zeolite catalysts and highlights the potential for broader application in commercial dehydrogenation processes and beyond.

关键词： Digital health   glucagon-like peptide-1 receptor agonists   population health   sodium-glucose cotransporter-2 inhibitors   type 2 diabetes  

摘要： Sodium-glucose cotransporter-2 inhibitors (SGLT2i) and glucagon-like peptide-1 receptor agonists (GLP1 RA) reduce cardiovascular and kidney risk in patients with type 2 diabetes (T2D), yet real-world use remains suboptimal. Remote care models offer a promising approach to improve access; there is limited data on the safety of and adverse events (AEs) associated with prescribing and titrating these therapies through a protocol-driven remote management program.

关键词： diamond spin chain   Haldane phase   fragmentation   magnon crystal   magnetocaloric cooling   quantum heat engine  

摘要： We investigate magnetic and thermodynamic properties of a spin-1 Heisenberg diamond chain in a magnetic field using a combination of analytical and numerical methods including the variational approach, exact diagonalization, density-matrix renormalization group, localized-magnon theory, and quantum Monte Carlo simulations. In the unfrustrated regime, the model exhibits a quantum ferrimagnetic phase that captures key magnetic features of the nickel-based polymeric compound [Ni3(OH)2(C4H2O4)(H2O)4] & centerdot;2H2O such as a flat minimum in the temperature dependence of the susceptibility times temperature product and an intermediate one-third magnetization plateau. In the frustrated regime, we uncover a rich variety of unconventional quantum phases including uniform and cluster-based Haldane states, fragmented monomer-dimer phase, and bound-magnon crystals. Analysis of the adiabatic temperature change and magnetic Gr & uuml;neisen parameter reveals an enhanced magnetocaloric effect near field-induced transitions between these exotic quantum phases. Additionally, we demonstrate that the frustrated spin-1 diamond chain can operate as an efficient working medium of a quantum Stirling engine, which approaches near-optimal efficiency when driven into these unconventional quantum states.

关键词： epilepsy   ferroptosis   heme oxygenase-1   hippocampus   hypoxia-inducible factor 1α   mitochondrial ultrastructure   oxidative stress   PX-478   reactive oxygen species   seizure behavior  

摘要： Epilepsy is a prevalent neurological disorder in which hippocampal neuronal damage,particularly ferroptosis,plays a critical *** studies have shown that hypoxia-inducible factor 1αis considered an important regulator of cellular stress responses and has been confirmed to play a critical role in the occurrence of various ***,the mechanisms by which hypoxia-inducible factor 1αis related to epilepsy and neuronal ferroptosis remain *** this study,we used a pentylentetrazole-induced chronic epilepsy mouse model and treated the mice with intraperitoneal administration of PX-478,a hypoxia-inducible factor-1α*** results showed that PX-478 significantly prolonged the latency of epilepsy,reduced seizure severity,and shortened seizure ***-478 also alleviated neuronal damage in the hippocampal CA1 and CA2 regions,reduced levels of reactive oxygen species and malondialdehyde,and increased levels of superoxide dismutase,catalase,and glutathione *** electron microscopy showed that PX-478 treatment reduced mitochondrial damage in the hippocampal neurons of epileptic mice,and significantly improved mitochondrial length and ***,PX-478 preferentially reduced Fe^(2+)levels and the expression of cyclooxygenase-2,ferritin heavy chain 1 and transferrin in the hippocampus of epileptic *** also inhibited the activity of the hypoxia-inducible factor 1α/heme oxygenase-1 *** summary,these findings suggest that PX-478 has the potential to treat epilepsy by inhibiting the hypoxia-inducible factor 1α/heme oxygenase-1 pathway,alleviating oxidative stress,and reducing ferroptosis in hippocampal neurons.

关键词： Lateral   Incision   Numbness   Arthroplasty  

摘要： A meta-analysis of randomised trials was conducted to compare clinical and functional outcomes between the standard anteromedial or midline skin incision and unconventional anterolateral incision for total knee arthroplasty (TKA).

关键词： APOE   Vascular graft   Fibrosis   Profibrotic macrophage   LRP1  

摘要： Vascular graft fibrosis can cause a decrease in cellular infiltration and capillary ingrowth in vascular walls. It can also lead to vascular stiffening. As such, there are still no vascular grafts that can be used in blood vessels where their diameters are less than 6 mm in patients. Although various approaches have been evaluated to mitigate implant-associated fibrosis, effective treatments remain quite limited. In this study, we demonstrated that Apolipoprotein E (APOE) significantly increased during vascular regeneration after graft implantation in vivo. APOE knockout (KO) increased compliance of the regenerated aortas, reduced extracellular matrix (ECM) deposition, and increased capillary ingrowth in adventitia of the regenerated aortas. Using single cell RNA sequencing (scRNA-seq), a subset of profibrotic macrophages was found to be involved in graft fibrosis. APOE KO reduced the formation of profibrotic macrophages during vascular regeneration. The interaction between APOE and low-density lipoprotein receptor related protein 1 (LRP1) partially mediated the profibrotic macrophage formation. The profibrotic macrophages promoted graft fibrosis mainly through secretion of insulin-like growth factor-1 (IGF-1) that could support fibroblast proliferation. Finally, we showed that APOE knockdown in vivo using adeno-associated virus (AAV) improved the compliance of the regenerated aortas, reduced extracellular matrix (ECM) deposition and increased capillary ingrowth in the adventitial areas of the regenerated aortas by reducing the formation of profibrotic macrophages and their secreted IGF-1. Collectively, these data indicate that APOE can promote profibrotic macrophage formation partially through LRP1, and the profibrotic macrophages mediate graft fibrosis by increasing fibroblast proliferation via IGF-1. Inhibition of APOE generation in regenerated aortas can alleviate graft fibrosis that occurs during vascular regeneration.

关键词： Mitochondrial metabolism   Oral squamous cell carcinoma   CMKLR1   Prognosis   Oxidative phosphorylation  

摘要： Oral squamous cell carcinoma (OSCC) is a common oral cavity malignancy with a poor prognosis. Although chemokine-like receptor 1 (CMKLR1) has been implicated in tumor progression in several types of cancer, its role and underlying mechanisms in OSCC remain unclear. In this study, we examined the role and mitochondrial metabolism pattern of CMKLR1 in OSCC progression.