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New tight junction protein 2 variant causing progressive familial intrahepatic cholestasis type 4 in adults: A case report

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World Journal of Gastroenterology 2020年第5期26卷 550-561页

作者： Chun-Shan Wei naja becher Jenny Blechingberg Friis Peter Ott Ida Vogel Henning Grønbæk Department of Clinical Medicine-Department of Hepatology and Gastroenterology Aarhus University HospitalAarhus N DK-8200Denmark Department of Hepatology Shenzhen Traditional Chinese Medicine HospitalThe Fourth Clinical Medical College of Guangzhou University of Chinese MedicineShenzhen 518033Guangdong ProvinceChina Department of Clinical Genetics Aarhus University HospitalAarhus N DK-8200Denmark

BACKGROUND Progressive familial intrahepatic cholestasis(PFIC)encompasses a group of autosomal recessive disorders with high morbidity and *** in the gene encoding tight junction protein-2(TJP2)have been linked to PFIC type 4(PFIC4),which predominantly presents in ***,there are only limited data from adults with TJP2-related *** report a family with an autosomal recessive disorder with a novel variant in the TJP2 gene in adults with very variable expression of *** SUMMARY The index patient presented at 19 years old with liver cirrhosis and variceal bleeding and was treated with endoscopic banding and *** 2018,he developed primary liver cancer that was treated with radiofrequency ablation followed by liver transplantation in *** testing revealed a novel homozygous TJP2 variant causing PFIC4(TJP2([NM_004817.3]:c.[3334C>T];[3334C>T])).The consanguineous family consists of the father and mother(both heterozygous)and their 12 children,of which five carry the variant in a homozygous state;however,these five siblings have highly variable expression of *** homozygous brothers had cirrhosis and portal hypertension at diagnosis at the ages of 19 and *** other homozygous brothers,age 23 and 19,and the homozygous sister,age 21,have elevated liver enzymes but presently no cirrhosis,which may suggest an age-dependent *** addition,five sisters had severe and mild intrahepatic cholestasis of pregnancy and carry the TJP2 variant in a homozygous and heterozygous state,*** This novel TJP2 variant is associated with PFIC4 causing severe liver disease with cirrhosis and primary liver cancer in adolescents/adults.

关键词： Progressive familial intrahepatic cholestasis Tight junction protein 2 Genetic variants Liver cirrhosis Liver cancer Case report

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Microstructures, hardening and tribological behaviors of tin matrix composites reinforced with SiC and Zn particles

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Rare Metals 2021年第9期40卷 2584-2592页

作者： Fatemeh Ghasemi Mohammad Moazami-Goudarzi Hamidreza najaﬁ Department of Materials Engineering Science and Research BranchIslamic Azad UniversityTehran ***Iran

In order to strengthen pure tin and improve its dry sliding resistance, Sn/SiC and Sn/Zn composites were fabricated via a powder metallurgy route. Microstructure,hardness and pin-on-disk wear resistance of pure tin and the fabricated composites were compared to those of Sn–7.5 Sb–3.5 Cu Babbitt alloy. The dominant wear mechanism at different applied loads was determined by analyzing worn surfaces and wear debris in each case. The results showed that the hardening effect of Zn was much higher than that of SiC. The hardening role of Zn in the tin matrix was ascribed to the direct load transfer mechanism. Microscopic studies of the worn surfaces revealed that the pure tin was susceptible to surface fatigue wear and plowing damage, depending on the normal load applied during the wear test. In the case of Sn/SiC composite and the Babbitt alloy, delamination wear mechanism resulting from subsurface crack propagation controlled the wear rate. While the highest hardness and the lowest coefficient of friction were obtained for the Babbitt alloy, the Sn/Zn composite exhibited the highest wear resistance at a constant applied load, indicating the importance of asperity contact type in the wear process.

关键词： Tin matrix composites Babbitt alloy Microstructure Hardening mechanisms Wear behavior

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Familial Mediterranean Fever: Clinical and Genetic Characteristics among Lebanese Pediatric Population

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Open Journal of Rheumatology and Autoimmune Diseases 2016年第3期6卷 63-73页

作者： Sirine Mneimneh Amal Naous Ziad naja Zeina naja Ahmad Salaheddine naja Andre Megarbane Mariam Rajab Department of Pediatrics Makassed General Hospital Beirut Lebanon Institut Jerome Lejeune Paris France

Objective: The aim of our study was to evaluate the clinical features, to define the frequency of mutation type, to assess genotype-phenotype correlation and the response to colchicine in childhood-onset Familial Mediterranean fever (FMF) in Lebanon. Methods: The characteristics of 550 children, presenting with FMF symptoms between January 2003 and January 2013 and having a positive Mediterranean fever gene (MEFV gene) mutation, were prospectively investigated. The clinical and genetic characteristics as well as the response to colchicine and its side effects were studied in 321 FMF children. The mutations were correlated with clinical presentation and disease severity. Results: Out of the 321 patients (183 males and 138 females), abdominal pain was the most common presenting feature documented in 84.7%. Mutational analysis detected simple heterozygotes, compound heterozygotes and homozygotes in 56.4%, 30.9% and 11.2% patients respectively. The most frequent mutation was M694V (37.2%), followed by E148Q mutation (27.4%). 71% patients received colchicine therapy;only 33.3% of them showed complete response. Genotype-phenotype correlation showed that M694V followed by E148Q was associated with moderate to severe disease form (71.6% and 62.7% respectively, P = 0.005). There was no association between mutation type and colchicine response. Conclusion: The most important features were the predominance of the M694V and E148Q. The M694V subgroup, followed by E148Q subgroup had a high disease severity score. Our data indicate an enhanced expression of the disease with E148Q mutation.

关键词： Familial Mediterranean Fever MEFV Gene Mutation Colchicine

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Flocculation of the Moroccan phosphates slimes

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Journal of Chemistry and Chemical Engineering 2009年第12期3卷 7-14页

作者： J. Briuni J. naja R. M＇Hamdi Natural Resources Valorization and Environment Laboratopy University of Science and Technology Settat 577 Morocco Water and Environment Laboratory Study and Research Center of Mineral Phosphates Casablanca 25679 Morocco

In Morocco, the phosphate industry generates by washing during the ore beneficiation in humid way, big quantities of sludge loaded in fine mineral particles （or slimes） with contents of water in the order of 90-95%. The specific consumption in water for the beneficiation of one tone of dry ore is approximately 0.6 m3. The management of this sludge and the recycling of process water are played to a schema of solid-liquid separation by flocculation, settling then stocking in basins of several dozens hectares. Recovered clear waters are recycled into the circuit of washing. The principal motivation of this work which is a part of EL MAA project is dedicated to the study of the impact of the speed of agitation on the flocculation behavior in terms of dewatering of the phosphate slimes and recycling of the process water. Study was driven on sample of slimes taken in entrance of the thickeners of a Moroccan washing plants, before flocculation. The raw slimes have a concentration in solid of 75.5 g·L^-1, a P205 grade of 17.3% and a free settling velocity 9 cm·h^-1. Fine fraction -2 μm, responsible for the poor settling of slimes is composed in practice of nearly 96% of smectite. It represents 9% of the dry solid content in the slimes. Measurements reveal important variations of the granulometric and textural properties of large floes in response to flocculation, opening ways of optimization for the velocity gradient.

关键词： flocculation phosphate slime water recycling

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Elimination of Cationic and Anionic Dyes by Natural Phosphate

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Journal of Chemistry and Chemical Engineering 2012年第8期6卷 721-725页

作者： A. Achkoun J. naja R. M'Hamdi Applied Chemistry and Environment Laboratory University of Science and Technology Settat 577 Morocco Water and Environment Laboratory Study and Research Center of Mineral Phosphates Casablanca 25679 Morocco

The present work aims to study the adsorption, determining the ability of phosphate to remove cationic dyes, MB （methylene blue） and CV （crystal violet） and an anionic dye, the MO （methyl orange）. To explain the important reduction of pollution parameters obtained from its use as a filter bed. Natural phosphate is contacted with an aqueous solution having a concentration of 20 mg/L for 90, 45 and 20 min respectively for the MB, CV and MO. Experimental results show that the phosphate totally eliminates the CV and the WB. While, the adsorption of MO is very low. The Langmuir isotherm applies to MB and MO while the adsorption of CV uses the Freundlich isotherm.

关键词： Natural phosphate adsorption isotherm methylene blue crystal violet methyl orange adsorption kinetics.

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Violence Prevention in a Psychiatric Unit

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Journal of Health Science 2014年第10期2卷 487-490页

作者： Ramzi Haddad Sami Richa Michelangelo Aoun Elyssar Ghaith Wadih naja Department of Psychiatry Campus des Sciences Medicales Damascus Road P. O. Box 11-5076 Riad El Solh Beirut 1107 2180Lebanon Department of Psychiatry Faculty of Medical Sciences Lebanese University Mount Lebanon Hospital Camille ChamounBoulevard P. O. Box 470 Hazmieh 1107 2090 Lebanon

Psychiatric units are one of the most frequent settings of violence against medical staff. This affects the physical, emotional and psychological well-being of the staff and has an impact on their quality of care and the interactions with patients inside the unit. Evaluate if SDAS scores at admission in an inpatient psychiatric unit can be predictive of violence and aggression among admitted patients towards medical staff. Charts of all patients admitted during a 12 month period were retrospectively reviewed and their SDAS scores at admission registered, and all violent events reported by nurses during the same period were collected. SDAS scores significantly contributed to the prediction of the violent behavior against nurses, non dependent on psychiatric diagnosis at admission. SDAS can represent a tool that can help psychiatric nurses identify patients at risk of violence and therefore improve management of these patients.

关键词： Violence aggression psychiatric unit psychiatric nurses.

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利用空间技术支持重大环境风险管理

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控制工程（北京） 1998年第6期 55-60页

作者： G.naja 殷书平

应用当前的空间技术和未来的空间技术支持环境风险管理具有巨大的开发潜力。这一应用领域具有无可置疑的社会效益和经济效益。此外,它还具有两个固有的重要特征:首先,这种应用是由航天界外部提出某项为满足用户(主要是民用环境保护当局... 详细信息

应用当前的空间技术和未来的空间技术支持环境风险管理具有巨大的开发潜力。这一应用领域具有无可置疑的社会效益和经济效益。此外,它还具有两个固有的重要特征:首先,这种应用是由航天界外部提出某项为满足用户(主要是民用环境保护当局)所确定的具体需求的独立倡议;其次,这种应用并不要求开发新的空间系统,至少不需要在短期或中期内开发新的空间应用系统,因为这种应用实质上是以现有的或已经计划中的空间系统的协调优化开发利用为基础的。

关键词：环境风险管理空间技术自然灾害

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第13届亚洲化学大会论文摘要—— Synthesis, Characterization and DFT Studies of Bis-4-methylpiperidiniummethyl-urea as Corrosion Inhibitors

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华东理工大学学报（自然科学版） 2010年第1期36卷 125-125页

作者： Teimouri A naja fi Chermahini A Payame Noor University （PNU） Isfahan PO Box 81395-671 Iran Department of Chemistry Yasouj University Yasouj Iran

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肺错构瘤的CT诊断及误诊分析(附12例报告)

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实用放射学杂志 1999年第12期 737-739页

作者：刘娜嘉刘芸马淑敏宁夏医学院附属医院放射科宁夏银川市第一人民医院CT室

目的:报告12例肺错构瘤CT特征,鉴别诊断及误诊原因。方法:回顾性分析12例经手术病理证实的错构瘤CT所见。结果:术前诊断正确者5例,误诊7例,分别误诊为:肺癌(周围型)、肺包虫、肺结核及包囊性积液。结论:瘤体内钙化并不是本病的特征性表... 详细信息

目的:报告12例肺错构瘤CT特征,鉴别诊断及误诊原因。方法:回顾性分析12例经手术病理证实的错构瘤CT所见。结果:术前诊断正确者5例,误诊7例,分别误诊为:肺癌(周围型)、肺包虫、肺结核及包囊性积液。结论:瘤体内钙化并不是本病的特征性表现,重视肿瘤的形态特点,注意显示病灶内脂肪结构是减少误诊提高定性诊断准确性的关键

关键词：肺错构瘤体层摄影,X线计算机

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颈丛阻滞治疗肌筋膜疼痛

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中国康复 2021年第7期36卷 422-422页

作者：夏楠(译) naja A 不详

由于引发肌筋膜性颈痛和/或肩痛的肌肉的支配神经均起源于颈丛,故本研究旨在评估颈丛深部神经阻滞对此类疼痛的治疗作用。研究同步采集了患者的年龄、病史、疼痛史、疼痛特点及先前治疗数据。受试者为患肌筋膜疼痛且无手臂放射症状的成... 详细信息

由于引发肌筋膜性颈痛和/或肩痛的肌肉的支配神经均起源于颈丛,故本研究旨在评估颈丛深部神经阻滞对此类疼痛的治疗作用。研究同步采集了患者的年龄、病史、疼痛史、疼痛特点及先前治疗数据。受试者为患肌筋膜疼痛且无手臂放射症状的成人,所有人在尝试口服止痛药、消炎药和物理治疗后均未能缓解。受试者随机分为两组,分别接受生理盐水注射安慰剂治疗和颈丛深部神经阻滞。阻滞注射物共7mL,其中包含3mL的2%的利多卡因和肾上腺素(1:200000)、3mL的0.5%布比卡因和1mL可乐定(150μg/mL)。使用VAS视觉疼痛模拟量表评估患者疼痛程度,并记录疼痛的持续时间。所有患者均接受相同的药理性疼痛治疗管理。研究最终纳入受试66例,其中实验组34例,安慰剂组32例。结果显示,治疗两周后,实验组与对照组患者的平均每日疼痛时长分别为1.38和5.25小时(P<0.001)。出现严重疼痛的情况在两组中的占比分别为2.9%和53.1%(P<0.0001)。结论:这项关于成人颈肩部肌筋膜疼痛的研究发现,颈丛神经阻滞可以显著改善此类患者的疼痛严重程度并减少疼痛持续时间。

关键词：神经阻滞生理盐水注射安慰剂实验组布比卡因颈丛颈痛消炎药

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