中国罕见病研究的现状与未来

罕见病是人类社会发展的沉重负担,对罕见病进行深入研究具有重要的社会意义和科学价值。中国人口基数巨大,为罕见病研究提供了广阔资源,同时也带来了巨大挑战。既往中国在罕见病领域取得了众多成就,同时也面临着研究力量分散、资源整合缺乏等问题。随着政府部门、学术机构、研究团体及个人对罕见病研究的重视及参与,中国罕见病研究正朝着协作、创新方向发展。未来的中国罕见病研究将从多个方面推进,为中国及世界应对罕见病挑战作出贡献。     

Bridging the critical interface: Ambidextrous innovation for water provision in Nairobi's informal settlements

This article examines innovation activities in water infrastructure in Nairobi, Kenya. The focus is on efforts by Nairobi City Water and Sewerage Corporation (NCWSC) to provide water services to the inhabitants in the informal areas of the city using an automated vending machine, or Pre-Paid Dispenser (PPD). In this study, we investigate what happens when a regime actor like NCWSC tries to implement an ambidextrous (two-handed) strategy: managing the existing system according to conventional practice and at the same time innovate in new technical solutions and business models to cater for unconnected users.Besides presenting our results, our aim in this article is also to introduce a research strategy for innovation studies in infrastructural systems in low-income urban areas. Three interconnected parts stand out as our contribution:First, we present a novel conceptual framework, by adding ideas from innovation studies to the Large Technical Systems approach. We investigate how regime actors can innovate to provide water to unconnected users, without expanding the system in a traditional way.Second, we propose a novel method for analysing and understanding innovation on the margins of the infrastructural system, or in our terms, innovation in the critical interface. Central to our method is, through empirical observation, to identify misalignment between the innovation (PPD) and contextual factors at the local level, where the innovation is implemented (interface misalignment), and misalignment between the innovation and the existing water regime (internal misalignment). We use a qualitative method and results forms as a basis for further research and starting point for regime actors in search of an improved ambidextrous strategy.Third, we analyse the innovation process per se. In the Nairobi case, we assess the ambidextrous innovation strategy, and claim that the PPD functions as an adapter in the critical interface, enabling the regime actors to operate in an environment of misalignment.     

关于运营商局房管理系统新增功能的浅析

本文对运营商局房资源管理现状进行了分析,阐述了资源管理系统增加审批模块及资源更新的方案,实现工作流程的有效监督。     

超临界CFB机组汽动给水泵油中进水分析

白马循环流化床示范电站600MW超临界CFB机组自投运以来,A、B汽动给水泵组经常出现润滑油质偏差现象,主要原因为油中含水过大造成。通过对给水泵相关系统进行全面的分析研究,指出了润滑油进水的原因是,给水泵轴承和密封水采用的呼吸器易堵塞造成回油室为较大负压状态。给水泵转速变化时,低压密封水室压力轻微波动,微正压的水进入微负压的回油室,造成油中微量带水。经过分析研究提出改进建议,运行后取得了较好效果,汽动给水泵组的油中含水量大幅降低,保证了机组安全稳定运行。     

Investigation on energy storage and conversion properties of multifunctional PANI-MWCNT composite

Polyaniline-multiwalled carbon nanotube (PANI-MWCNT) composite synthesized through chemical polymerization is investigated as a possible electrode material for supercapacitor as well as an electro-catalyst for hydrogen evolution reaction (HER) in acidic medium. UV–Vis spectroscopy, FTIR spectroscopy and field emission scanning electron microscopy (FESEM) have been used to characterize the electrode material. The binder-free electrodes were prepared and they exhibit a specific capacitance of 540.29 F g^?1 at a scan rate of 2 mV s^?1 in 1 M H₂SO₄ electrolyte. The material exhibits excellent pseudocapacitive behaviour due to the presence of PANI with long-term cyclic stability of 87.4% retention after 5000 cycles. PANI-MWCNT composite also shows good HER activity, with overpotential of ?395 mV.     

Fault handling in large water networks with online dictionary learning

Fault detection and isolation in water distribution networks is an active topic due to the nonlinearities of flow propagation and recent increases in data availability due to sensor deployment. Here, we propose an efficient two-step data driven alternative: first, we perform sensor placement taking the network topology into account; second, we use incoming sensor data to build a network model through online dictionary learning. Online learning is fast and allows tackling large networks as it processes small batches of signals at a time. This brings the benefit of continuous integration of new data into the existing network model, either in the beginning for training or in production when new data samples are gathered. The proposed algorithms show good performance in our simulations on both small and large-scale networks.     

Compiler compatible 5.66 Mb/mm2 8T 1R1W register file in 14 nm FinFET technology

1-read/1-write (1R1W) register file (RF) is a popular memory configuration in modern feature rich SoCs requiring significant amount of embedded memory. A memory compiler is constructed using the 8T RF bitcell spanning a range of instances from 32 b to 72 Kb. An 8T low-leakage bitcell of 0.106 μm² is used in a 14 nm FinFET technology with a 70 nm contacted gate pitch for high-density (HD) two-port (TP) RF memory compiler which achieves 5.66 Mb/mm² array density for a 72 Kb array which is the highest reported density in 14 nm FinFET technology. The density improvement is achieved by using techniques such as leaf-cell optimization (eliminating transistors), better architectural planning, top level connectivity through leaf-cell abutment and minimizing the number of unique leaf-cells. These techniques are fully compatible with memory compiler usage over the required span. Leakage power is minimized by using power-switches without degrading the density mentioned above. Self-induced supply voltage collapse technique is applied for write and a four stack static keeper is used for read Vmin improvement. Fabricated test chips using 14 nm process have demonstrated 2.33 GHz performance at 1.1 V/25 °C operation. Overall V_min of 550 mV is achieved with this design at 25 °C. The inbuilt power-switch improves leakage power by 12x in simulation. Approximately 8% die area of a leading 14 nm SoC in commercialization is occupied by these compiled RF instances.     

Co3O4 nanoparticles decorated Polypyrrole/carbon nanocomposite as efficient bi-functional electrocatalyst for electrochemical water splitting

An effective bi-functional electrocatalyst of Co₃O₄/Polypyrrole/Carbon (Co₃O₄/Ppy/C) nanocomposite was prepared through a simple dry chemical method and used to catalyze the oxygen evolution reaction (OER) and hydrogen evolution reaction (HER). Three types of carbon support as Vulcan carbon, reduced graphite oxide (RGO) and multi-walled carbon nanotubes (MCNTs) were used to study the influence on electrochemical reactions. Spherical shaped Co₃O₄ nanoparticles with 8–10 nm was found uniformly distributed on Ppy/C composite, which were analyzed by X-ray diffraction and transmission electron microscopy techniques. Amongst, Co₃O₄/Ppy/MWCNT shows improved bifunctional electrocatalytic activity towards both OER and HER with relatively low over potential (340 mV vs. 490 mV at 10 mA cm⁻²) and Tafel slope (87 vs. 110 mV dec⁻¹). In addition to that, MWCNT supported Co₃O₄/Ppy nanocomposite exhibits good electronic conductivity and electrochemical stability up to 2000 potential cycles. The results clearly indicate that the Co₃O₄/Ppy/MWCNT nanocomposite could be the promising bi-functional electrocatalyst for efficient water electrolysis.     

A study on the Fe–Cl thermochemical water splitting cycle for hydrogen production

Thermochemical water splitting cycles are recognized as one of the promising pathways for sustainable hydrogen production. In the present study, Iron-chlorine (Fe–Cl) cycle as one of the chlorine family thermochemical cycles where iron chloride is consumed for hydrogen production from water, is considered for a study. This four-step cycle is modelled by Aspen Plus software package and analyzed for performance investigation of each reaction step and system's components. The parametric studies are also performed to assess the effect of operation conditions such as temperature, pressure and steam to feed ratio on the reaction products and conversion rates. Results indicated that although the effect of pressure is not significant on reaction's production rates, an increase in temperature favors oxygen production in reverse deacon reaction and magnetite production in hydrolysis and lowers hydrogen production in the hydrolysis step. On the other hand, steam to chlorine (Cl₂) ratio is directly correlated with hydrochloric acid (HCl) and oxygen production in reverse deacon reaction and hydrogen production in hydrolysis.     

NaFeTiO4: A novel visible light active photocatalyst for water splitting and environmental remediation

Single phase, crystalline NaFeTiO₄ with tunnel structure is prepared by a solid state method and explored as a novel photocatalyst for the first time. Structural, optical and morphological properties of NaFeTiO₄ are investigated by various characterization techniques such as X-ray diffraction (XRD), scanning & transmission electron microscopy (SEM & TEM), Energy dispersive X-ray spectroscopy (EDS), N₂ adsorption-desorption study (BET), UV-vis, X-ray photoelectron, X-ray absorption (UV-vis DRS, XPS and XANES) and photoluminescence (PL) spectroscopy. The interfacial charge transfer ability of the prepared n-type NaFeTiO₄ was also investigated by transient photocurrent response and electrochemical impedence spectroscopy which proved to be an efficient tool for better understanding of electronic properties of NaFeTiO₄. The photocatalytic efficiency of NaFeTiO₄ is evaluated for decomposition of methylene blue (MB) and Rhodamine B (RhB) dyes as well as for H₂ evolution through water splitting reaction under visible light. NaFeTiO₄ exhibits efficient charge separation properties, excellent photocatalytic activities and reusability.