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患儿 男，11月龄，以频繁成簇抽搐发作起病，予地西泮、苯巴比妥及丙戊酸钠联合治疗无效，每天抽搐发作数十次，起病第4天因癫痫持续状态入住重症监护病房治疗，并出现嗜睡、意识模糊、不能认人、不自主动作、偶兴奋尖叫、夜间睡眠时间减少，脑电图背景活动慢化，监测到频繁后头部起源的临床发作及电发作，脑脊液抗AMPA2受体IgG抗体阳性，诊断抗AMPA2受体脑炎。予人免疫球蛋白及激素冲击免疫治疗，加用拉考沙胺并减停丙戊酸后抽搐缓解，意识转清。临床上以频繁局灶抽搐发作伴有意识水平下降为主要表现的婴儿，应注意鉴别抗AMPA2受体脑炎，尽早启用免疫治疗改善预后至关重要。.

ALDH4A1, a member of the aldehyde dehydrogenase superfamily, is a key enzyme in the mitochondrial proline metabolism pathway. Recent studies have shown that mutations in aldh4a1 lead to reduced fertility and reproductive premature aging of male nematodes. However, the effect of ALDH4A1 on fertility of male mice has not been studied. In this study, we used CRISPR-Cas9 technology to construct a knockout mouse model of Aldh4a1 for the first time to explore the effect of this gene on the reproduction of male mice. The results showed that compared with WT male mice, Aldh4a1^(-/-) male mice were fertile, had normal spermatogenesis but defect in sperm maturation in the epididymis documented by impaired motility, increased morphological abnormalities and increased spontaneous acrosome reaction. In addition, transmission electron microscopy showed vacuoles in the sperm mitochondria, and fracture in the neck of sperms and vacuoles in these mice. These results revealed that ALDH4A1 plays a vital role in the structure of sperm flagellum and the process of sperm maturation in mice.

To study the molecular mechanism of interferon-alpha (IFN-α) in the treatment of hepatocellular carcinoma (HCC) and the molecular markers that can predict the therapeutic effect, differentially expressed (DE)-miRNAs, -mRNAs, -lncRNAs, and -circRNAs were screened between 12 samples collected from 4 patients who had not received treatment (control), 4 patients who had received recombinant human interferon a-2b treatment (case1), and 4 patients who had relapsed after receiving recombinant human interferon a-2b treatment (case2). Enrichment analyses were performed to determine the principal functions of the DE-RNAs. We also constructed protein-protein interactions (PPI) and competing endogenous RNA (ceRNA) networks. In addition, a series-cluster analysis was performed to analyze changes in gene expression across different groups of HCC. Furthermore, the expression of the genes were verified in the Cancer Genome Atlas (TCGA) database. A total of 36 union DE-miRNAs, 175 union DE-mRNAs, 65 union DE-lncRNAs, and 52 union DE-circRNAs were obtained between the control vs case1, and case2 vs case1 groups. DE-mRNAs were mainly involved in the mitochondrial inner membrane. DE-circRNAs were mainly enriched in the Golgi apparatus. ceRNA network contained 68 DE-mRNAs, 26 DE-miRNAs, 45 DE-lncRNAs, and 23 DE-circRNAs. A total of 24 DE-miRNAs, 175 DE-mRNAs, 65 DE-lncRNAs, and 52 DE-circRNAs were classified into eight profiles, respectively. A total of 26 genes showed a significant correlation with prognosis of HCC (p0.05). Some genes may be used to predict the efficacy of IFN-α in the treatment of HCC. The results may lay a foundation for investigating the different sensitivities of IFN-α in the treatment of HCC.

2例长节段脊髓磁共振成像信号改变的Leigh病患儿分别为3岁男性和8岁女性。分别于2018和2020年就诊于广州市妇女儿童医疗中心。主诉分别为“左下肢乏力半年余，右下肢乏力2个月”和“双下肢乏力5个月余伴排尿困难1周”。2例患儿分别存在MT-ND4基因m.11470AC变异和MT-ND5基因m.13514AG变异。经线粒体鸡尾酒疗法治疗后，2例患儿病情改善。.

患儿男，11岁，因发热、头痛于2018年5月就诊广州市妇女儿童医疗中心，抗感染治疗无效，病程中出现行走、独坐不稳，精神行为异常，病原学检查阴性，磁共振成像（MRI）双侧基底节多发异常信号，软脑膜及脊膜强化，脑脊液转染小鼠脑组织免疫荧光染色发现胶质纤维酸性蛋白（GFAP）抗体，诊断自身免疫性胶质纤维酸性蛋白星形胶质细胞病。予静脉输注甲泼尼龙冲击治疗后，临床症状改善，上述病灶吸收，无神经系统后遗症。.

Magnesium alloys are lap-joined to galvanised and bare steel sheets by a cold metal transfer method. The weld appearance, cross-section, tensile strength and fracture behaviour of these joints are characterised by scanning electron microscopy, tensile tests and energy-dispersive spectroscopy. The joints were found to have good weld appearance and satisfactory tensile strength. The spreadability and wettability of the Mg alloy–galvanised steel joint are superior to those of the Mg alloy–bare steel joint, but the tensile strength is lower. In particular, the presence of Zn on the galvanised steel sheet improves wettability but decreases tensile strength. Aluminium has a high affinity for Fe, and the thinner layer of Fe–Al improves the mechanical properties of the Mg alloy–bare steel joint.

Supplemental Material for Differences in clinico-pathological characteristics and outcomes between proteinase 3-ANCA positivity and myeloperoxidase-ANCA positivity in lupus nephritis by C Li, J -J Wang, M -L Zhou, D -D Liang, J Yang, H -X Zhu, W -X Hu and H -T Zhang in Lupus

The electronic structure, magnetism properties and optical absorption of organometal halide perovskite CH3NH3XI3 (X = Fe, Mn) are studied using the first principles calculations by the generalized gradient approximation (GGA) and the GGA + U method, respectively. The magnetic ground states of CH3NH3MnI3 and CH3NH3FeI3 are both the G-type antiferromagnetic (AFM) order. With the introduction of on-site Coulomb interactions U, CH3NH3FeI3 shows the semiconducting phase from original metallic state predicted by the GGA method. The band gap value of CH3NH3MnI3 with the G-AFM state is 1.68 eV, while the band gap in the spin majority channel is 0.694 eV and the band gap in the spin minority channel is 2.147 eV when system is in FM state. For CH3NH3FeI3 system, the band gap is 0.957 eV when system is in G-AFM state, while the band gap in the spin majority channel is 0.602 eV and the band gap in the spin minority channel is 1.215 eV when system is in FM state, which shows that photo-excited electrons of CH3NH3MnI3 and CH3NH3FeI3 with FM state will rapidly melt the local magnetic order. Furthermore, the optical properties of CH3NH3MnI3 and CH3NH3FeI3 are calculated. CH3NH3MnI3 with the FM state shows strong infrared light absorption. CH3NH3FeI3 with FM state have stronger infrared absorption than that in G-AFM state.

The widespread availability of Ultrasound machines and their relatively low cost and functionality make them an attractive tool to use during the treatment of ongoing Crohn's Disease (CD). This study aims at exploring the value of conventional, power Doppler and contrast-enhanced ultrasound during the active stages of CD.24 patients in the active stages of Crohn's disease were enrolled in the study. The full, medial and lateral intestinal wall thicknesses and the thickness ratio of medial to lateral intestinal wall of the segmental lesions were measured by conventional ultrasound. The diseased intestinal wall was also examined by power Doppler ultrasound to assign Limberg classification types: 3 cases were Limberg II, 9 cases were Limberg III and 12 cases were Limberg IV type. Importantly, the full and medial thicknesses of the intestinal walls with different Limberg types were compared, and statistically significant differences were found (p0.05). Finally, images of the diseased segments were taken by contrast-enhanced ultrasound, and the contrast agent bolus arrival, the inflow and the peak enhancement times were calculated in order to be able to distinguish intestinal wall thickness differences according to different Limberg types.Cases with Limberg types III and IV mostly showed total intestinal enhancement, while Limberg II type cases showed mostly medial intestinal enhancement. When comparing the inflow and peak times of contrast-enhanced ultrasound of patients with different Limberg types, the differences found were statistically significant (p0.05).This study confirms incrassation of the intestinal wall being the main ultrasonic appearance of active CD. Both power and contrast-enhanced ultrasound are effective tools in the management of active CD: while power ultrasound can be used to carry out Limberg typing, contrast-enhanced ultrasound can analyze and diagnose incrassation segments of the intestinal wall with different disease stages.

Platelets are megakaryocyte-derived nuclear-free fragments that participate in cardiovascular diseases including acute myocardial infarction, ischemic stroke, hypertension, and atherosclerosis. At the endothelium damage site, platelets interact with sub-endothelial matrix proteins such as glycoprotein VI/Fc receptor γ-chain (GPVI/FcRγ), G protein-coupled receptor/phospholipase Cγ(β) (GPCR/PLCγ(β)), Rho/RhoK and integrin. The activation of these signaling pathways triggers intracellular calcium increase and causes platelet adhesion, aggregation, granule release and finally thrombus formation. Some endogenous platelet modulators reported to negatively regulate this process are: (1) platelet surface inhibitory receptors: carcinoembryonic antigen cell adhesion molecule 1, 2 (CEACAM 1, 2), platelet endothelial cell adhesion molecule-1 (PECAM-1), and G6b-B; (2) nuclear receptors: retinoic X receptor (RXRs), glucocorticoid receptor (GR), peroxisome proliferator-activated receptors (PPARs) and liver X receptors (LXRs); (3) intracellular adaptor proteins: CLP36, paxillin, downstream of tyrosine kinase (Dok), c-Casitas B-lineage lymphoma (c-Cbl), protein kinase Cδ (PKCδ), glycogen synthase kinase(GSK)-3β, phospholipase D2 (PLD2), peroxiredoxin II (PrxII), T-cell ubiquitin ligand-2 (TULA-2); (4) extracellular modulators released from platelet granules: adapter protein disabled-2 (DAB2) and diadenosine 5,5-P1, P2-diphosphate (Ap2A). The discovery of biological or endogenous modulators of platelet activation is regarded as a potential therapeutic target for thrombotic disease. This review highlights the recent findings on the endogenous negative regulatory molecules released from platelets and their impact on platelet thrombus formation.

Using the first principles calculations, the electronic and optical properties of C, Mo and Mo-C-doped anatase TiO2 are studied. For the Mo mono-doped TiO2, the band gap reduces little, and the largest perturbation occurs at the CBM of TiO2. C mono-doping suppresses the effective band gap, but the partially occupied subbands in the gap probably also serve as the recombination centers for electrons and holes. Therefore, the Mo-C co-doping is investigated for the charge compensation consideration. We discuss six doped configurations and find that the total energy of the system is increased with increasing distance of C and Mo. It is found that co-doped configurations with C nearest to Mo possess the lowest total energy. Then, we focus on discussing three possible Mo-C adjacent co-doped configurations. The subbands mainly induced by C-2p states in the band gap become fully occupied because the Mo atom contributes sufficient electrons to C anion for compensation. At the same time, the effective band gap is narrowed about 0.9 eV and the perturbation at the CBM occurred in Mo mono-doped TiO2 disappears, which means the band edges of doped system still straddle the redox potentials of water. Furthermore, the optical properties of the compensated Mo-C adjacent co-doped TiO2 and pure TiO2 are calculated. The optical absorption edges of the Mo-C co-doped TiO2 shift towards the visible light region.

AbstractBiomorphic TiC/C ceramics with a wood-like structure were synthesised using a template fabricated from wood. TiC coatings were obtained on the surface of a carbon template via reacting it with Ti powder in a mixture of a molten KF–KCl binary salt under an argon atmosphere. Phase composition and microstructure of the TiC/C ceramics were characterised by X-ray diffraction, scanning electron microscopy and X-ray spectroscopy. The pore size distribution within the porous TiC/C ceramics was investigated using automatic mercury porosimetery. The results demonstrated that Ti powder reacted with the cellular carbon walls of the wood to form TiC coatings at 700–1000°C. Furthermore, the synthesised TiC/C ceramic retained the morphology of the carbon template, indicating that the formation of the carbon template played a dominant role in the formation of the TiC/C ceramic possessing a highly microporous structure.

5-hydroxytryptamine receptor 1A (5-HT1AR) is closely associated with cognitive functions. Selective serotonin reuptake inhibitors (SSRIs) can protect individuals from brain damage following ischemia/hypoxia. To investigate the function of SSRIs in vascular dementia (VD), we established a rat model of VD, and observed the effect of SSRIs on the expression of 5-HT1AR mRNA and neurotransmitters. Male SD rats (6 months) were randomly assigned into sham, model, and SSRI groups (N = 30). VD was achieved by permanent ligation of the bilateral common carotid artery. Escitalopram, a highly selective 5-HT reabsorption inhibitor, was ip injected into the rats for three consecutive weeks. The Morris water-maze was used to test learning and memory. H&E staining for neuronal injury was conducted on cortical and hippocampal tissues. HPLC was used to determine the levels of dopamine (DA), 5-HT, and norepinephrine (NE). RT-PCR was used to determine expression of 5-HT1AR mRNA. As compared to control rats, model animals demonstrated elongated escape latency, lower platform crossing times, and significant injuries to hippocampal CA1 neurons. This was accompanied by reductions in DA, 5-HT, and NE levels in hippocampal tissues, as well as reduced cortical 5-HT and decreased 5-HT1AR mRNA expression (P < 0.05). Escitalopram treatments reduced escape latency, elevated platform crossing times, improved CA1 neuronal damage, increased DA and 5-HT levels in hippocampal and cortical neurons, as well as elevated expression of 5-HT1AR mRNA (P < 0.05). Therefore, SSRIs may improve cognitive dysfunction of VD rats, possibly by stimulating expression of neurotransmitters and protecting neurons.

The electronic structure and optical absorption spectrum of organometal halide perovskite compound CH3NH3BiI3 as a substituting candidate of well-concerned CH3NH3PbI3 not only for environmental friendly consideration are studied using the first principles calculations. It is revealed that a Bi replacement of Pb in CH3NH3PbI3 does not change seriously the band edge structure but the bandgap becomes narrow. Consequently, CH3NH3BiI3 exhibits not only stronger visible light absorption than CH3NH3PbI3 does but more strong absorption in the infrared region, which is however absent in CH3NH3PbI3. It is suggested that CH3NH3BiI3 may be one of even more promising alternatives to CH3NH3PbI3 for spectrum-broad and highly-efficient solar cells.

In this study, cold metal transfer (CMT) plug welding of 1 mm thick Mg AZ31 to 1 mm thick hot-dipped galvanized mild steel (i.e., Q235) was studied. Welding tests were performed and the process variables optimized with Mg AZ61 wire and 100% argon shielding gas for a plug weld located in the center of the 25 mm overlap region. It was found that it is feasible to join 1 mm thick Mg AZ31 workpiece to 1 mm thick galvanized mild steel using CMT plug welding. The optimized process variables for CMT plug welding Mg AZ31-to-galvanized mild steel were a wire feed speed of 10.5 m/min, a predrilled hole with a diameter of 8 mm in Mg AZ31 workpiece and a welding time of 0.8 s. CMT plug welded Mg AZ31-to-galvanized mild steel joints were composed of the fusion zone between Mg AZ31 base metal and Mg weld metal, Mg weld metal (i.e., combined base metal, filler wire and Zn coating), and the brazing interface between magnesium weld metal and galvanized mild steel. The brazing interface mainly consisted of Al, Zn, Mg, Si intermetallic compounds and oxides (i.e., Fe3Al, Mg2Si, MgZn, and MgZn2), and magnesium solid solution. The static strength of CMT welded-brazed Mg AZ31-galvanized steel was determined primarily by the strength and area of the brazed interface and thickness of the intermetallic reaction layer.

The electronic structure and optical absorption spectrum of organometal halide perovskite compound CH3NH3BiI3 as a substituting candidate of well-concerned CH3NH3PbI3 not only for environmental friendly consideration are studied using the first principles calculations. It is revealed that a Bi replacement of Pb in CH3NH3PbI3 does not change seriously the band edge structure but the bandgap becomes narrow. Consequently, CH3NH3BiI3 exhibits not only stronger visible light absorption than CH3NH3PbI3 does but more strong absorption in the infrared region, which is however absent in CH3NH3PbI3. It is suggested that CH3NH3BiI3 may be one of even more promising alternatives to CH3NH3PbI3 for spectrum-broad and highly-efficient solar cells.

In this paper, Ni–Co–TiO2 composite electrodeposits were successfully prepared on AZ91D magnesium alloy for protecting the alloy from corrosion. Scanning electron microscopy, X-ray diffraction and energy dispersive spectroscopy were employed to investigate the surface morphologies, microstructure and chemical compositions of the composite coatings respectively. The electrochemical corrosion behaviours of the coatings in neutral 3·5 wt-%NaCl, 0·6 mol L−1 Na2SO4 and 0·6 mol L−1 NaOH solutions were evaluated by electrochemical impedance spectroscopy and potentiodynamic polarisation techniques, and the results indicate good protection for AZ91D magnesium alloy. Long term immersion tests were carried out in neutral 3·5 wt-%NaCl solution using electrochemical impedance spectroscopy, showing that Ni–Co–TiO2 composite coating could provide long term protection in neutral 3·5 wt-%NaCl solution for AZ91D magnesium alloy.

Polydimethylsiloxane (PDMS) is widely used in the electrical and electronic industries due to its excellent electrical insulation and biocompatible characteristics. However, the dielectric constant of pure PDMS is very low which restricts its applications. Herein, we report a series of PDMS/Al2O3/strontium titanate (ST) composites with high dielectric constant and low loss prepared by a simple experimental method. The composites exhibit high dielectric constant (relative dielectric constant is 4) after the composites are coated with insulated Al2O3 particles, and the dielectric constant gets further improved for composites with ST particles (dielectric constant reaches 15.5); a lower dielectric loss (tanδ= 0.05) is also found at the same time which makes co-filler composites suitable for electrical insulation products, and makes the experimental method more interesting in modern teaching.

We have designed a new type of composite materials which not only has a Young's modulus much larger than the Voigt limit, but also is always nearly isotropic. Moreover, its Poisson's ratio can be designed at a desired value, e.g. positive, or negative, or zero. We have also demonstrated that structural hierarchy can help to enhance the stiffness of this type of composite materials. The results obtained in this paper provide a very useful insight into the development of new functional materials and structures.

NiAl have been fabricated by reactive sintering compacts of ball-milled powder mixtures containing Ni and Al. The reaction mechanism, as well as phase and microstructural development, were investigated by analyzing compacts quenched from different temperatures during reactive hot compaction. It was found that the reaction process was strongly affected by pressure, heating rates, heat loss from the sample to the environment. The application of 50 MPa prior to the reaction resulted in the intermetallic-formation reaction initiating at a temperature (480°C) much lower than that (∼550°C) when no pressure was applied. At high heating rate (50°C/min), when the heat loss is small, the formation of NiAl occurs rapidly via combustion reaction. On the other hand, if the heat loss is significant as in slow heating rate (10°C/min), the reaction process is controlled by solid-state diffusion. The phase formation sequence for the slow solid-state reaction was determined to be: NiAl3→ Ni2Al3→ NiAl → NiAl (Al-rich) + Ni3Al → NiAl.

Water is necessary for sustaining human life. In many regions of the world, traditional water sources cannot meet increasing water demands. Warm-cloud seeding is an efficient way to augment water supplies. In this paper, we explore two new hygroscopicity catalysts: Poly acrylamide (PAM) and Sodium polyacrylate (PAAS). We designed a series of experiments to investigate the effects of these catalysts together with common catalyst salt powder (NaCl). The experiment was held in a cloud chamber built in our laboratory. The results show that: 1) Catalysed by NaCl, a dose of 0.91g/m3 can obtain the best precipitation efficiency and enhancement rate at 70.8% and 142%, respectively; 2) A 1.36g/m3 dose catalysed by PAM and PAAS exhibits optimal performance at 76.7% and 70.4% precipitation efficiency, respectively; 3) Under the same conditions, PAM shows better catalytic effects than NaCl does.

This article focuses on the relation between laccase-secreting ability and growing cycle in the edible fungi. First, laccase activities of fifteen different edible fungi were detected and determined by plate assay and spectrophotometric method using 2,2′-azinobis(3-ethylbenzothiazoline-6-sulfonic acid) as the substrate. The results showed the laccase-secreting ability in the edible fungi and their growing cycles are closely related. The edible fungi strains with short growing cycles originate from their high levels of secreted laccase activity. However, those strains require long growing cycles due to the low levels of secreted laccase, even no detectable laccase activity. The research provides the first evidence on the corresponding relation between the level of secreted laccase activity and growth cycles of edible fungi. Our study has significantly increased the understanding of the role of laccase in the growth and development of edible fungi.

The tensile response of oriented polyethylene and polystyrene films provides material data for the modelling of closed-cell polymer foams. We analyse a Kelvin foam, with 60% of the polymer in the cell faces, compressed in the [001] direction. Cell edges bend and compress axially, while cell faces act as membranes. The tensile strains across some face diagonals are 40% of the foam compressive strain, so tensile yielding can occur. The predicted Young’s moduli are slightly low, because compressive face stresses are ignored, but the Poisson’s ratio is correctly predicted. The predicted yield stresses for polyethylene foams are close to experiment. Polystyrene extruded foams may collapse in compression when face yielding commences.