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Clinical and genetic analysis of nonketotic hyperglycinemia:A case report

World Journal of Clinical Cases 2022年第22期10卷 7982-7988页

作者： Jun-Jie Ning Feng li sheng-qiu li Department of Pediatric Intensive Care Unit First People's Hospital of Zigong CityZigong 643000Sichuan ProvinceChina

BACKGROUND Nonketotic hyperglycinemia(NKH)is a rare autosomal recessive genetic disorder of abnormal glycine metabolism caused by insufficient activity of the glycine cleavage enzyme *** is believed to function mainly as an inhibitory neurotransmitter,but it can also act as a co-agonist of the N-methyl-D-aspartate(NMDA)*** accumulation of a large amount of glycine in the brain leads to neuronal and axonal injury via overactivation of NMDA receptors located in the hippocampus,cerebral cortex,olfactory bulb,and cerebellum and to stimulation of the inhibitory function of glycine receptors located in the spinal cord and brain stem,resulting in central apnea,hiccups,and hypotonia in the early stage of the *** SUMMARY The child described in this report had typical clinical manifestations of NKH,such as hiccups,disturbance of consciousness,hypotonia,and convulsions,within the first week after ***-exome genetic testing revealed that the child had a compound heterozygous mutation,namely,c.395C>A(p.S132X)and c.2182G>A(p.G728R),in the GLDC gene,and he was diagnosed with *** treatment,we administered an oral levetiracetam solution and added topiramate and prednisone for epilepsy control,but the epilepsy remained *** diet therapy was started at 6 mo of age,his seizures were significantly reduced,and there were no obvious adverse reactions during ketogenic ***,we found that with the development of the disease,high levels of serum glycine decreased or even disappeared without intervention,and as the disease progressed,the corpus callosum became *** This case shows that plasma glycine levels cannot be used to evaluate the prognosis of NKH,that the development of the corpus callosum can be affected by NKH,and that a ketogenic diet may be effective for seizure control in NKH patients.

关键词： Nonketotic hyperglycinemia Compound heterozygosity GLDC gene Case report

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Disseminated Fusarium bloodstream infection in a child with acute myeloid leukemia:A case report

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World Journal of Clinical Cases 2021年第21期9卷 6049-6055页

作者： Jun-Jie Ning Xue-Mei li sheng-qiu li Department of PICU First People's Hospital of Zigong CityZigong 643000Sichuan ProvinceChina

BACKGROUND Disseminated Fusarium is rare in healthy *** with hematological tumors may have secondary fungal infections,including Fusarium infections,which are due to tumor bone marrow infiltration or prolonged bone marrow suppression after *** of the lack of typical clinical manifestations and effective antifungal drugs,early diagnosis and treatment of the disease are difficult,and the prognosis is *** SUMMARY The patient in this case was a 13-year-old female child with rash and fever as the first *** had the characteristics of the four stages of skin that are typical of Fusarium *** was diagnosed with disseminated Fusarium infection through skin biopsy and blood culture and diagnosed with Fusarium solani infection based on the morphological characteristics of the blood *** treatment with liposome amphotericin B combined with voriconazole,the child *** This case highlights that for children with secondary agranulocytosis after receiving chemotherapy for hematological malignancies,once typical abnormal skin damage is found,the possibility of Fusarium infection should be considered,and voriconazole alone or in combination with polyenes may be the most effective anti-Fusarium *** B,the traditional drug of disseminated Fusarium disease,has a high mortality rate,and it is not recommended to use it *** neutrophil counts are essential for the treatment of disseminated Fusarium bloodstream infection.

关键词： Fusarium liposomal amphotericin B Voriconazole Acute myeloid leukemia Agranulocytosis Case report

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EXPERIMENTAL STUDY ON A NEW TYPE OF AERATOR IN SPILLWAY WITH LOW FROUDE NUMBER AND MILD SLOPE FLOW

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Journal of Hydrodynamics 2009年第3期21卷 415-422页

作者： SU Pei-lan liAO Hua-sheng qiu Yue li Chen-juan State Key Laboratory of Hydraulics and Mountain River Engineering Sichuan University Chengdu 610065 China

Experimental study on aeration characteristics of various aeration devices was conducted in the spillway tunnel of the Pubugou hydropower project, Sichuan Province, China. It is shown by comparison that the new type of aeration device, namely, the aerator with a trapezoidal-shaped slot and a steep-slope section（ATSS）, can avoid water accumulation in the cavity of the aeration device in the project, thus can effectively solve the backwater problems arising from this project and be used for a wide range of different water levels, without any drain facilities. Above the water level of 840 m, the water contained in the cavity can be eliminated completely, which means that the recommended new type of aerator can meet the aeration demands in the spillway of the project with low Froude number and may be of practical significance and of interest to other projects with similar types of aeration devices.

关键词： aeration device a trapezoidal-shaped slot and a steep-slope section(ATSS) air entrainment cavitation

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Fe/MCM-41 as a promising heterogeneous catalyst for ozonation of p-chlorobenzoic acid in aqueous solution

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Chinese Chemical Letters 2011年第6期22卷 683-686页

作者： Rui Huan Huang Jie liu Lai sheng li qiu Yun Zhang li Xuan Zeng Ping Lu School of Chemistry & Environment South China Normal University Guangzhou 510006 China

Mesoporous MCM-41 and Fe loaded MCM-41（Fe/MCM-41）,which were successfully prepared by a hydrothermal method and a dipping method respectively,were applied as heterogeneous catalysts for ozonation of p-chlorobenzoic acid（p-CBA） in aqueous ***-41 and Fe/MCM-41 were characterized by XRD,FT-IR and diffuse reflectance UV-vis（DR-UV-vis） techniques. The presence of either MCM-41 or Fe/MCM-41 improves p-CBA and total organic carbon（TOC） removal efficiency compared to ozonation *** the experimental condition,TOC removal rate of Fe/MCM-41/O_3 process is over 63.5%at 60 min oxidation time,44.5%using MCM-41 as catalyst,only 37.7%with ozonation *** presence of tert-butanol（TBA） in the Fe/MCM-41/ O_3 process indicated that the oxidation mechanism of p-CBA occurs via OH in the liquid *** Fe/MCM-41 is a promising catalyst.

关键词： Fe/MCM-41 Catalytic ozonation p-Chlorobenzoic acid TOC

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试论中古早期西欧的“王权神授”

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河南大学学报（社会科学版） 2010年第4期50卷 88-92页

作者：邱胜利河南大学历史文化学院河南开封475001

中古早期西欧"王权神授"的神权政治理念起源于基督教的《圣经》之中,在基督教的神权政治文化传统中,蕴涵着浓厚的"王权神授"的政治理念,"王权神授"的理论是通过教会为国王施涂油加冕礼而具体地表现出来的,它赋予了世俗权力从未有过的神... 详细信息

中古早期西欧"王权神授"的神权政治理念起源于基督教的《圣经》之中,在基督教的神权政治文化传统中,蕴涵着浓厚的"王权神授"的政治理念,"王权神授"的理论是通过教会为国王施涂油加冕礼而具体地表现出来的,它赋予了世俗权力从未有过的神圣,但也给它加上了从未有过的束缚。涂油加冕典礼为以后罗马教会的教权高于俗权的主张提供了口实,同时也为教权与俗权之争埋下了隐患。

关键词：中古早期西欧王权神授涂油加冕典礼

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20世纪中期以来西方中世纪英国王权研究

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山西师大学报（社会科学版） 2011年第5期38卷 85-89页

作者：邱胜利河南大学历史文化学院河南开封475001

长期以来,中外学者对中世纪英国王权的性质和地位存在较大的分歧,至今尚未形成广泛的共识。20世纪中期以来,西方学者从宪政、契约、法理、财政等不同的角度出发,对中世纪英国王权进行了系统研究,并取得了重要的研究成果,但成熟的理论体... 详细信息

长期以来,中外学者对中世纪英国王权的性质和地位存在较大的分歧,至今尚未形成广泛的共识。20世纪中期以来,西方学者从宪政、契约、法理、财政等不同的角度出发,对中世纪英国王权进行了系统研究,并取得了重要的研究成果,但成熟的理论体系尚未建立。有的学者认为王权受到制约,王权没有公权;也有的学者则强调国王的公共政治权威。争论的焦点在于王权是否受到来自法律的限制,或者说多大程度上受到限制,王权是有限的还是专制的。这些研究有助于我们更好地理解中世纪英国王权的性质和地位。

关键词： 20世纪中期中世纪英国王权

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基于欧拉多相流模型的桥梁主梁三维风驱雨数值研究

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工程力学 2017年第4期34卷 63-71页

作者：刘顺黄生洪李秋胜陈伏彬湖南大学土木工程系湖南长沙410082 中国科学技术大学工程科学系安徽合肥230026 香港城市大学建筑学与土木工程学系香港999077 长沙理工大学土木与建筑学院湖南长沙410114

该文利用欧拉多相流模型,对矩形断面和准流线型断面的桥梁主梁进行了三维风驱雨数值模拟计算。通过计算,得到了桥梁主梁周围风雨流场的分布和主梁上雨滴平均冲击荷载分布,并重点探讨了降雨对桥梁主梁断面三分力系数的影响以及来流攻角... 详细信息

该文利用欧拉多相流模型,对矩形断面和准流线型断面的桥梁主梁进行了三维风驱雨数值模拟计算。通过计算,得到了桥梁主梁周围风雨流场的分布和主梁上雨滴平均冲击荷载分布,并重点探讨了降雨对桥梁主梁断面三分力系数的影响以及来流攻角和湍流强度的变化对风驱雨效应的影响。通过和相关风洞试验数据的比较,验证了欧拉多相流方法在桥梁工程风驱雨研究中的有效性和便利性,为桥梁工程风驱雨研究提供了一个新的手段。通过分析发现,降雨会影响桥梁主梁的三分力系数,但雨滴对主梁的平均冲击荷载相对于风荷载而言较小,影响并不显著;攻角和湍流强度的变化对风驱雨效应的影响亦不明显。

关键词：欧拉多相流模型桥梁主梁风驱雨冲击荷载三分力系数攻角湍流强度

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