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新生儿ANK3基因突变致先天发育异常1例

中华实用儿科临床杂志 2022年第7期37卷 543-545页

作者：牟鑫王又平冯晋楠武辉吉林大学第一医院新生儿科长春130000

对吉林大学第一医院新生儿科2019年5月确诊的1例伴ANK3基因杂合突变的先天发育异常患儿的临床资料进行回顾性分析。患儿,女,10 min,因"出生窒息,复苏后10 min"就诊,患儿呈特殊外貌,自主呼吸少,无吞咽功能,肌张力极低,原始反射未引出,振... 详细信息

对吉林大学第一医院新生儿科2019年5月确诊的1例伴ANK3基因杂合突变的先天发育异常患儿的临床资料进行回顾性分析。患儿,女,10 min,因"出生窒息,复苏后10 min"就诊,患儿呈特殊外貌,自主呼吸少,无吞咽功能,肌张力极低,原始反射未引出,振幅整合脑电图(aEEG)提示暴发抑制(BS)背景活动,BS(+),下边界2μV,上边界50μV,无睡眠觉醒周期,未见惊厥发作,原始脑电图暴发段见较多机械样刷,印象:重度异常aEEG。采用高通量测序基因检测发现患儿基因存在ANK3基因c.4183(exon33)C>G及c.8239(exon37)C>T杂合突变,均未见报道。本病例丰富了ANK3基因突变的临床表型,有利于对该基因的认识与研究。

关键词：先天发育异常 ANK3基因常染色体隐性精神智力发育迟滞37型婴儿,新生基因突变

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九道河隧洞软岩地层洞段变形控制措施研究

水利建设与管理 2022年第1期42卷 25-30页

作者：王有平云南省滇中引水工程建设管理局楚雄分局云南楚雄675000

软岩隧洞变形是西南地区隧洞开挖的常见难题之一。本文围绕九道河隧洞工程,针对Ⅳ类和Ⅴ类围岩洞段,通过分析软弱围岩的地质特征,详细探讨了软弱围岩隧道施工处理措施。九道河隧洞工程遵循"保护围岩、岩变我变,支护宁强勿弱,二衬及早跟... 详细信息

软岩隧洞变形是西南地区隧洞开挖的常见难题之一。本文围绕九道河隧洞工程,针对Ⅳ类和Ⅴ类围岩洞段,通过分析软弱围岩的地质特征,详细探讨了软弱围岩隧道施工处理措施。九道河隧洞工程遵循"保护围岩、岩变我变,支护宁强勿弱,二衬及早跟进"的原则,充分调动围岩的承载能力,有效控制了围岩变形和松弛,最终确保了隧道施工的顺利进行。其成功经验,可为同类地质条件下的隧道施工提供借鉴。

关键词：隧道软岩地层变形控制施工技术

共产主义学说对乌托邦的批判——兼论马克思与黑格尔的理想主义共识

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思想与文化 2023年第1期32卷 212-222页

作者：王有凭东南大学人文学院

因未详陈理想社会的建设性内容,马克思(Karl Marx)的共产主义学说往往被自由主义学者判定为乌托邦。但结合黑格尔(***)关于理想社会的论述可知:其一,马克思在批判乌托邦的前提下强调共产主义并非纯然道德意义的社会,而是扬弃资本主义社... 详细信息

因未详陈理想社会的建设性内容,马克思(Karl Marx)的共产主义学说往往被自由主义学者判定为乌托邦。但结合黑格尔(***)关于理想社会的论述可知:其一,马克思在批判乌托邦的前提下强调共产主义并非纯然道德意义的社会,而是扬弃资本主义社会的历史必然进程;其二,马克思对乌托邦的批判借鉴了黑格尔理性国家理论的“事后反思”;其三,马克思描述未来理想社会时运用了黑格尔历史目的论。共产主义与理性国家具有亲缘性,二者皆为扎根于人类历史与社会现实生活的理想主义表达。

关键词：乌托邦共产主义理想主义 “事后反思” 历史目的论

3'-Sialyllactose regulates glucose and lipid metabolic disorders via the gut–liver axis in mice fed a high-fat die

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Food Science and Human Wellness 2024年

作者： Wei Zhang Meizhen Zhu Kelsang Dekyi Linxi Zheng Yichen Zhang youping Lv Dongbei Guo Xiaoxuan Chen Lili Pan Xinyue wang Hongwei Li State Key Laboratory of Vaccines for Infectious Diseases， Xiang An Biomedicine Laboratory， School of Public Health， Xiamen University State Key Laboratory of Molecular Vaccinology and Molecular Diagnostics，National Innovation Platform for Industry-Education Integration in Vaccine Research， Xiamen University Department of Nutrition， Zhongshan Hospital (Xiamen)， Fudan University Xiamen Clinical Research Center for Cancer Therapy

Recent research has indicated that sialic acid， such as free sialic acid (N-acetylneuraminic acid， Neu5Ac) and bound sialic acid (3'-sialyllactose， 3'-SL)， can ameliorate disorders associated with glycolipid metabolism， although the underlying mechanisms have yet to be determined. We examined the effects of 3'-SL on glycolipid metabolism in mice fed a high-fat diet. Male C57BL/6J mice were divided into six groups: two model control groups (normal and high-fat diets) and four intervention groups [Neu5Ac， and low-，moderate-， and high-dose 3'-SL]. After 8 weeks of continuous gavage intervention， mice in the 3'-SL intervention groups had lower body weight and total fat content; reduced fasting blood glucose， triglycerides， low-density lipoproteins and oxidized-low-density lipoproteins; and increased high-density lipoproteins，but no dosage-dependent of 3'-SL intervention was found， 3’-SL＿M intervention as optimal for further exploration. 3'-SL intervention could increase respiratory exchange ratio， energy expenditure， and amount of exercise performed. 3'-SL increased the colonic abundances of Akkermansia， Lactobacillus， and Bacteroides， and reduced those of Erysipelatoclostridium， Faecalibaculum， and Aldercreutzia. Changes were also observed in colonic metabolites， and liver gene transcript and metabolites， which were mainly enriched in bile secretion， taurine and hypotaurine metabolism， and insulin resistance. Additionally， 3'-SL was observed to regulate genes associated with physiological rhythms， including clock， per2， cry1， and bhihe41. Collectively， our findings indicate that 3'-SL can contribute to the prevention and control of disorders associated with glucose and lipid metabolism caused by high-fat diets. Compared with Neu5Ac， 3'-SL intervention can ameliorate intestinal flora disorders， enhance bile acid circulation， increase tissue energy expenditure， and reduce lipid synthesis， thereby promoting lipid-lowering effects mediated via the liver–gut axis， and can a

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Review on Hydroxyurea Usage in Young Children with Sickle Cell Disease: Examining Hemoglobin Induction, Potential Benefits, Responses, Safety, and Effectiveness

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International Journal of Clinical Medicine 2024年第1期15卷 1-18页

作者： Maiko Charles Mkwambe youping Deng Dongchi Zhao Department of Pediatrics Zhongnan Hospital of Wuhan University Wuhan China

Sickle cell disease (SCD) is a prevalent condition, particularly in the countries of sub-Saharan Africa, where the presence of specific genes associated with Malaria contributes to its high prevalence. Patients with sickle cell disease frequently experience painful episodes necessitating hospitalization, and their hemoglobin levels are typically lower than those of the general population. There are different treatment options available to manage complications, such as transfusing blood, hydroxyurea, and strong anti-pains. However, with all these treatments, patients still commonly experience pain crises and suffer from organ damage. Hydroxyurea, the sole approved medication for sickle cell anemia in developed and developing countries, is widely used in children despite being primarily indicated for adults. Multiple studies have demonstrated the efficacy of hydroxyurea in inducing HbF production in young children with SCD. Elevated HbF levels have been associated with improved clinical outcomes, including a reduction in vaso-occlusive crises, acute chest syndrome, and the need for blood transfusions. Furthermore, increased HbF levels have been shown to ameliorate disease-related organ damage, such as pulmonary hypertension and sickle cell retinopathy. The response to hydroxyurea treatment in young children with SCD is variable. Some patients achieve substantial increases in HbF levels and experience significant clinical benefits, while others may have a more modest response. Factors influencing the response include baseline HbF levels, genetic modifiers, treatment adherence, and dose optimization. Safety is a crucial consideration when using hydroxyurea in young children. Studies have shown that hydroxyurea is generally well-tolerated, with the most common adverse effects being myelosuppression, gastrointestinal symptoms, and dermatological manifestations. However,long-term effects and potential risks, such as renal dysfunction and reproductive impacts, require furth

关键词： Effectiveness Hydroxyurea Sickle Cell Disease Sickle Cell Anemia Minimally Effective Dose Maximum Tolerated Dose

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华北晚更新世的石片石器

人类学学报 2019年第4期38卷 525-535页

作者：王幼平北京大学中国考古学研究中心北京大学考古文博学院

本文首先简要梳理了晚更新世华北地区石片石器发展历程;进而探讨石片石器与南邻的砾石工业,北部边疆地区新发现的莫斯特文化遗存,以及更晚来自西北方向的石叶/细石器技术的交流互动。在此基础上,重点讨论晚更新世华北石片石器与现代人... 详细信息

本文首先简要梳理了晚更新世华北地区石片石器发展历程;进而探讨石片石器与南邻的砾石工业,北部边疆地区新发现的莫斯特文化遗存,以及更晚来自西北方向的石叶/细石器技术的交流互动。在此基础上,重点讨论晚更新世华北石片石器与现代人在该地区出现与发展历程之间的关系,提出石片石器是华北地区旧石器文化发展的主流,亦是追溯该地区现代人出现与发展课题至关重要的考古学证据。

关键词：华北晚更新世石片石器现代人起源

Recent Advances for Global Perspectives on Etiology, Pathophysiology, Clinical Presentations, and Management of Moyamoya Disease

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World Journal of Neuroscience 2024年第1期14卷 6-23页

作者： Maiko Charles Mkwambe Dongchi Zhao youping Deng Department of Pediatrics Zhongnan Hospital of Wuhan University Wuhan China

Moyamoya disease (MMD) is a condition characterized by the gradual narrowing and blockage of blood vessels in the brain, specifically those in the circle of Willis and the arteries that supply it. This results in reduced blood flow and oxygen to the brain, leading to progressive symptoms and potential complications. The underlying pathophysiological mechanism remains elucidated. However, recent studies have highlighted numerous etiologic factors: abnormal immune complex responses, susceptibility genes, branched-chain amino acids, antibodies, heritable diseases, and acquired diseases, which may be the great potential triggers for the development of moyamoya disease. Its clinical presentation has varying degrees from transient asymptomatic events to significant neurological deficits. Moyamoya disease (MMD) shows different patterns in children and adults. Children with MMD are more susceptible to ischemic events due to decreased blood flow to the brain. Conversely, adults with MMD are more prone to hemorrhagic events involving brain bleeding. Children with MMD may experience a range of symptoms including motor impairments, sensory issues, seizures, headaches, dizziness, cognitive delays, or ongoing neurological problems. Although adults may present with similar clinical symptoms as children, they are more prone to experiencing sudden onset intraventricular, subarachnoid, or intracerebral hemorrhages. One of the challenges in moyamoya disease is the potential for misdiagnosis or delayed diagnosis, particularly when physicians fail to consider MMD as a possible cause in stroke patients. This review aims to provide a comprehensive overview of recent global studies on the pathophysiology of MMD, along with advancements in its management. Additionally, the review will delve into various surgical treatment options for MMD, as well as its rare occurrence alongside atrioventricular malformations. Exciting prospects include the use of autologous bone marrow transplant and the p

关键词： Moyamoya Disease (MMD) Etiology Pathophysiology Clinical Presentations Management Future Promising Avenues

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法治现代化视野下《刑事诉讼法》再修改

政法论坛 2024年第1期42卷 57-66页

作者：谢佑平湖南大学法学院

《刑事诉讼法》的再修改,应该坚持和恪守《刑事诉讼法》的中国特色与自主知识体系,加强和完善相关制度的政治性、人民性、专业性和民主集中性。认识和尊重《刑事诉讼法》的个性与刑事司法规律,关注刑事诉讼法的国际法属性,确立程序法定... 详细信息

《刑事诉讼法》的再修改,应该坚持和恪守《刑事诉讼法》的中国特色与自主知识体系,加强和完善相关制度的政治性、人民性、专业性和民主集中性。认识和尊重《刑事诉讼法》的个性与刑事司法规律,关注刑事诉讼法的国际法属性,确立程序法定、无罪推定原则和沉默权制度,完善控辩平等与控审分离,建立以审判为中心的诉讼制度。直面和回应刑事司法实务中的热点难点问题:健全立案监督机制,防范错误立案;细化管辖规定,阻断逐利执法;建立被害人国家补偿制度;废除指定居所监视居住,完善强制措施;将法援律师纳入司法责任制主体;将认罪认罚从宽定位为诉辩协商制度;设立单位犯罪合规案件特别程序;完善录音录像和证据制度;明确二审案件开庭审理要求;规范诉讼期限延长制度。

关键词：刑事诉讼法修改程序法定原则以审判为中心刑事司法规律法典化

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华北旧石器晚期环境变化与人类迁徙扩散

人类学学报 2018年第3期37卷 341-351页

作者：王幼平北京大学中国考古学研究中心北京100871 北京大学考古文博学院

近年来大量考古新发现与研究成果显示,受到地理区位与MIS3阶段以来气候变化双重影响,华北地区的本地文化与新来者持续移动扩散。本地的简单石核-石片技术经过长期发展,并不断与新来者交流融合,演进为以船型细石核技术为特色的华北旧石... 详细信息

近年来大量考古新发现与研究成果显示,受到地理区位与MIS3阶段以来气候变化双重影响,华北地区的本地文化与新来者持续移动扩散。本地的简单石核-石片技术经过长期发展,并不断与新来者交流融合,演进为以船型细石核技术为特色的华北旧石器晚期文化。来自欧亚大陆西侧的莫斯特与石叶技术,亦对华北地区有显著影响。MIS3阶段,外来影响仅表现为在高纬度地区向东扩散。应对MIS2阶段的环境压力,石叶与楔形细石核技术先后南下。华北地区原住民则可能是通过便于高流动性活动的船型细石核技术等适应方式,更好地维系了生存发展。

关键词：环境变迁人类迁徙石器技术深海氧同位素阶段3和2 华北