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HLA-C端粒端150kb区域与犹太人群银屑病相关

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世界核心医学期刊文摘（皮肤病学分册） 2006年第8期2卷 19-20页

作者： j.wish population martnez-Borra j. BrautbarC. González S. C. López-Larrea 朱国兴 Department of Immunology Hospital Universitario Central de Asturias 33006 Oviedo Spain

The HLA-Cw 0602 has been associated with psoriasis in different ethnic groups. But, it remains unclear whether HLA-C is the PSORS1 gene (the psoriasis gene in the MHC). Thus, several case-control studies have been performed in order to investigate whether HLA-C itself determines the susceptibility to the disease. We studied 59 j.wish patients with type I psoriasis and 79 matched controls. Polymorphic genes and markers from HLA-B (centromeric to HLA-C) to the corneodesmosin (CDSN) gene (telomeric to HLA-C) were genotyped in order to determine their contribution to the susceptibility to psoriasis. Neither HLA-Cw 0602 nor the allele CDSN TTC were significantly associated with psoriasis with the size of the sample studied. The genes and markers telomeric to HLA-C such as the microsatellite C1-4- 4 (OR = 2.6, 95% CI = 1.4- 4.7, Pc =0.018) the octamer transcription factor (OTF)- 3 gene (OR = 2.6, 95% CI = 1.6- 4.3, Pc = 0.0001) and the α -helix coiled-coil rod homologue (HCR) gene (OR = 2.5, 95% CI = 1.3- 4.5, Pc = 0.004), however, were associated with the disease. These results suggest that a maj.r psoriasis susceptibility gene is likely to be located within a region of 150 kb telomeric to HLA-C and centromeric to the CDSN gene.

关键词：种族人群银屑病 HLA-Cw*0602 端粒病例对照研究 RS1基因多态性基因基因分型等位基因易感性

Genotypes and Phenotypes of Uygur Children with Xeroderma Pigmentosum: A Case Study in Xinj.ang, China

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International j.urnal of Dermatology and Venereology 2024年第3期7卷 144-149页

作者： Qiu Y. Wang W.-j. Liang j.-Q. Yakeya B. Wang P. Zhang j.-Z. Kang X.-j. Department of Dermatology and Venereology People's Hospital of Xinjiang Uygur Autonomous Region Xinjiang Uygur Autonomous Region Urumqi 830001 China Xinj.ang Clinical Research Center for Dermatologic Diseases Xinjiang Uygur Autonomous Region Urumqi 830001 China Xinj.ang Key Laboratory of Dermatology Research (Xj.S1707) Xinjiang Uygur Autonomous Region Urumqi 830001 China

Obj.ctive: Xeroderma pigmentosum (XP) is a rare autosomal recessive dermatosis caused by genetic defects of DNA repair. This study was performed to detect and analyze the genes of 2 Uygur patients with XP and their families and assess the patients' phenotypes, which may enrich the understanding of the genetic skin disorder spectrum in Xinj.ang area. Methods: We collected the clinical data from 2 patients with XP and peripheral blood samples from the patients and their family members. The patients' DNA was sequenced and detected by Sanger sequencing, and gene mutations were screened. Results: The proband in family 1 presented with brown maculae at the exposure site and squamous cell carcinoma secondary to a facial rash. The proband had a homozygous nucleotide variation of XPC c.2251-2A> G (A change from A to G in the penultimate position of the intron before the 2251 position in the coding region), which was a shear mutation. In this family, both parents were heterozygous, and no similar mutation detected in the sister. In family 2, the proband had scattered black brown spots and papules on the trunk and limbs. and his younger sister was also a patient. The proband and his younger sister had homonucleotide variation of XPA c.631C> T, which was nonsense mutation, resulting in the codon for Arg No.211 being changed into termination codon (***211X), thus terminating the peptide chain synthesis prematurely. All the normal individuals in the two families were heterozygotes, and homozygous mutations occurred in all the patients, which was consistent with the autosomal recessive inheritance. Conclusion: XP is rare in Uygur population. This study expanded the mutation spectrum of XP and provided a basis for early diagnosis, treatment, prognostic prediction, and prenatal genetic consultation. © 2022 Hospital for Skin Diseases (Institute of Dermatology), Chinese Academy of Medical Sciences, and Chinese Medical Association, published by Wolters Kluwer, Inc.

关键词： gene mutation xeroderma pigmentosum XPA gene XPC gene

Wind power potential over northern South America using ERA5-Land global reanalysis

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CLEAN ENERGY 2024年第2期8卷 104-112页

作者： Arregoces, Heli A. Bonivento, Guillermo j. Roj.no, Roberto Univ Guaj.ra Fac Ingn Grp Invest GISA Riohacha Colombia Univ Antioquia UdeA Fac Ingn SIU Grp Proc Fisicoquim Aplicados Calle 70 52-21 Medellin Colombia

The northernmost region of South America has great potential for wind energy production and is a strategic area for the energy transition. We analysed data from ERA5-Land global reanalysis from 1980 to 2020 to determine the wind power potential in this area. Our aim was to identify areas with a high potential for wind energy that could be used to supply energy in case of drought in the region or increases in fuel prices. We compared data from 19 meteorological stations located in Colombia with ERA5-Land reanalysis data to generate reliable information for the feasibility and operation of wind farms. According to our analysis, the Colombian Caribbean and Coastal Plain have monthly average wind speeds at 90 m ranging from 4.85 to 10.34 m/s and 3.79 to 6.62 m/s, respectively. Based on wind power density calculations, these regions have the highest wind power density in our study, with a monthly average of between 250 and 399 W/m2. The Caribbean Region had a 69% increase in wind power density from j.nuary to j.ly compared with the annual average. The wind power density in the plains increased from j.nuary to March, while the Mountain Region had higher anomalies from May to October. The Alta Guaj.ra and Paraguana Peninsula have great potential to generate wind energy due to their privileged geographical location. Although ERA5 offers valuable information about wind availability in time and space, it cannot represent the influence of local effects on wind patterns for the Mountain Region. Data from ERA5-Land global reanalysis (1980-2020) are used to determine the wind power potential in the northernmost region of South America to identify areas with a high potential for wind energy, which could be used to supply energy in case of drought or increases in fuel prices. Graphical Abstract

关键词： onshore wind power wind power potential ERA5-Land global reanalysis wind speed ENERGY COLOMBIA REGION ERA5

Editorial for the Special Issue on Emerging Technology and Advanced Application of Nondestructive Detection for Power Equipment

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Chinese j.urnal of Electrical Engineering 2024年第1期10卷 1-2页

作者： Li j. Mei H. Li S. Tianj.n University China Tsinghua Shenzhen International Graduate School China Lanzhou j.aotong University China

The rapid development of clean and renewable energy technologies represented by wind and solar energy,as well as the continuous growth of load demand,have put forward higher requirements for the safe operation of the power *** performance of power equipment based on traditional dielectric materials is often limited by various defects generated during molding,transportation,assembly,and operation,which cannot meet the reliability requirements of power systems in the new *** detection is an essential technical method in industrial development,which can detect and evaluate the defect and damage status of in-service equipment,key components,and materials without damaging the obj.ct.

关键词： service Application operation

Exploring public perception and willingness to pay for a renewable source of energy: evidence from an emerging economy of renewable-energy development

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CLEAN ENERGY 2024年第2期8卷 177-188页

作者： Bashir, Mohammed j. K. Abdelhak, Senadj.i Salah, Wael A. Abuhelwa, Mai Cent Queensland Univ Sch Engn & Technol 120 Spencer St Melbourne Qld 3000 Australia Univ Tunku Abdul Rahman Fac Business & Finance Kampar Perak Malaysia Palestine Tech Univ Kadoorie Coll Engn & Technol Dept Elect Engn POB 7 Yafa St Tulkarm Palestine Univ Missouri Dept Elect Engn & Comp Sci Columbia MO 65211 USA

The study focuses on the transition from conventional energy sources to sustainable renewable energy (RE) in Palestine, particularly in the West Bank and Gaza Strip. The research aims to understand the relationship between key factors and residents' willingness to invest in the RE sector, with a specific focus on the impact of education. Using the unified theory of acceptance and use of technology, the study analyzes data from 400 residents through a questionnaire and employs ordinary least-squares regression analysis with Eviews 12. The key findings indicate that residents' inclination to invest in RE is significantly influenced by factors such as resource availability, knowledge level and power outages. Moreover, the study suggests that educated residents are more likely to invest in RE when factors such as knowledge, effort expectation, enabling conditions and resource availability are relatively high. The conclusion emphasizes the importance of policymakers' allocating sufficient funds to research and development to promote technological innovation in RE sources, thereby enhancing the population's energy efficiency. The study focuses on the transition from conventional energy to sustainable renewable energy in Palestine, particularly in the West Bank and Gaza Strip, to understand the relationship between key factors and residents' willingness to invest in the RE sector, with a specific focus on the impact of education. Graphical Abstract

关键词： renewable energy technology energy security public perceptions sustainable development UNIFIED THEORY RURAL-AREAS TECHNOLOGY ACCEPTANCE DETERMINANTS INTENTION RELIABILITY SERVICES SYSTEM ERRORS

Use of ketamine for severe asthma exacerbation in a pregnant patient with persistent bronchospasm: A case report

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Emergency and Critical Care Medicine 2024年第3期4卷 130-133页

作者： j.hnson M. Abbas M. Abdo j. Mansour M. j.nes j. Pharmacy Department Beaumont Health Dearborn MI United States Emergency Center Beaumont Health Dearborn MI United States

Background Estimated 4%-8% of pregnancies are complicated by asthma. Adequate management in this population is critical to minimize complications. Patients presenting with asthma exacerbation are typically managed with standard bronchodilators and systemic corticosteroids. However, additional agents may be used in patients with refractory asthma exacerbation. Ketamine has been used in refractory bronchospasm, although its efficacy in published literature is heterogeneous. Case Presentation We present a case of a pregnant patient with severe asthma exacerbation refractory to standard and salvage treatment who achieved termination of bronchospasm with ketamine infusion. Conclusion After receiving ketamine infusion for several days, the patient had improved air flow and achieved successful extubation, without experiencing rebound bronchospasm. Although this individual clinical case alone cannot change guidelines or directives to use in refractory asthma exacerbations, it offers a possible treatment option to patients and providers in unusually severe cases with extenuating risk factors. © Wolters Kluwer Health, Inc. All rights reserved.

关键词： Asthma Bronchospasms Case Report Ketamine Pregnancy

A hydrodynamic modelling of wave-activated bodies for wavelet marine regions

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CLEAN ENERGY 2024年第2期8卷 35-47页

作者： Peh, j. S. Muhieldeen, Mohammed W. Shuaib, N. H. UCSI Univ Fac Engn Technol & Built Environm Mech Engn Dept Kuala Lumpur 56000 Malaysia MTC Engineer Sdn Bhd Shah Alam Selangor Malaysia

Recently, most wave energy research has targeted sea areas with high power density under extreme wave conditions for deployment. This trend often leads to higher energy costs for wave power plants. However, many marine areas with low power density, characterized by wavelet conditions and less destructive forces than extreme wave conditions, remain underexplored. Therefore, this study aimed to propose a wave-activated body model for sea regions with wavelet conditions. The wave-activated body design process encompassed site selection, parameter determination, geometry design, comparison and performance evaluation using the ANSYS (R) AQWA model. The results indicated that the proposed device achieved the desired heave motion, with an amplitude range of 1.2 to -2.5 m, validating its potential for deployment in marine regions with wavelet conditions. Notably, while the proposed design is optimized for wavelet conditions, it was found to have potential limitations in extreme wave environments. This observation emphasizes the challenge of formulating a generalized design suitable for both conditions. Consequently, it is pivotal for wave-activated body designs to be customized based on the specific ocean conditions they target, underscoring the need for specialized designs of wave energy converters that consider the unique wave characteristics of their deployment sites. A wave-activated body is designed for energy generation in ocean regions with wavelet conditions. Hydrodynamic modelling is carried out for two locations with different wave characteristics to determine the optimum conditions for deployment. Graphical Abstract

关键词： wave energy converter South China sea renewable and sustainable energy wavelet ANSYS AQWA hydrodynamic wave-activated bodies ENERGY IMPACT

Early Syphilis Presenting as Thickened Penile Plaques With an Embedded Recurrent Ulcer (Chancre Redux): A Case Report

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International j.urnal of Dermatology and Venereology 2024年第3期7卷 160-162页

作者： Davies S.C. Hannaford R. Cotter j. Northern Sydney Sexual Health Service Northern Sydney Local Health District St Leonards 2065 NSW Australia Northern Beaches Dermatology Mona Vale 2103 NSW Australia

Introduction: The manifestations of syphilis are varied, and serology can establish the diagnosis early, especially in rare cases. We report a case of chancre redux (a rare recurrence of primary syphilis), which was embedded within a secondary syphilitic penile skin plaque. There were missed opportunities at earlier diagnosis as serology for syphilis was not ordered. Case presentation: A 56-year-old man presented with thickened penile plaques for five months. He reported a small penile ulcer approximately one month prior. There was no history of other skin lesions or rash. However, clinically there was an asymptomatic, indurated ulcer embedded within a plaque which was swab-positive for syphilis by PCR. A punch biopsy of a plaque was spirochaete-positive using an immunoperoxidase stain. The patient's lesions resolved three weeks after treatment with intramuscular benzathine penicillin. Discussion: Uncommonly reported even in the preantibiotic era, chancre redux is now rare. The ulcer usually recurs at or near the site of the original chancre, and has similar morphological features. The skin lesions of secondary syphilis can exhibit remarkable morphological variety. The most common skin manifestation, a generalized macular rash, was not present at any time in this case. Rather, there were only a couple of nonspecific penile plaques. If biopsy is performed, histologic findings are variable, though typically the inflammatory infiltrate includes plasma cells. A special immunoperoxidase stain can highlight spirochaetes in biopsy sections. Conclusion: This case highlights the importance of considering syphilis in the differential diagnosis of persistent, atypical penile lesions and underscores the need for appropriate serological testing in such instances. Copyright © 2023 Hospital for Skin Diseases (Institute of Dermatology),

关键词： chancre redux genital rash immunohistochemistry immunoperoxidase primary syphilis secondary syphilis

Error field penetration in j.TEXT tokamak based on two-fluid drift-MHD model

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Chinese Physics B 2024年第4期33卷 545-551页

作者：王文徐涛张仪 the j.TEXT team State Key Laboratory of Advanced Electromagnetic Technology International Joint Research Laboratory of Magnetic Confinement Fusion and Plasma PhysicsSchool of Electrical and Electronic EngineeringHuazhong University of Science and TechnologyWuhan 430074China

An externally generated resonant magnetic perturbation can induce complex non-ideal MHD responses in their resonant *** have studied the plasma responses using Fitzpatrick's improved two-fluid model and program *** calculated the error field penetration threshold for *** addition,we find that the island width increases slightly as the error field amplitude increases when the error field amplitude is below the critical penetration ***,the island width suddenly j.mps to a large value because the shielding effect of the plasma against the error field disappears after the *** scanning the natural mode frequency,we find that the shielding effect of the plasma decreases as the natural mode frequency ***,we obtain the m/n=2/1 penetration threshold scaling on density and temperature.

关键词： plasma responses drift-MHD model error field penetration