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Identification of compound heterozygous mutations in the ITGA2B gene in a Chinese patient with Glanzmann thrombasthenia

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Chinese Medical Journal 2010年第11期123卷 1397-1401页

作者： ZHENG Jia-yong JIN Yan-hui zhu yong-lin JIN Pei-pei ZHANG De-ting JIN Zi-bing Department of Laboratory Medicine Third People's Hospital ofWenzhou Wenzhou Zhejiang 325000 China Wenzhou Third Clinical Institute of Wenzhou Medical College Wenzhou Zhejiang 325200 China Diagnosis Center of Clinical Laboratory First Affiliated Hospitalof Wenzhou Wenzhou Zhejiang 325000 China Department of Clinical Transfusion Ruijin Hospital MedicalCollege of Shanghai Jiao Tong University Shanghai 200025China Eye Ear Nose and Throat Hospital of Fudan University Shanghai200031 China

Background Glanzmann thrombasthenia （GT） is an autosomal recessive bleeding disorder characterized by the tendency to hemorrhage and the inability of platelets to aggregate in response to agonists. GT is caused by a defect of the platelet glycoprotein lib/Ilia complex. The objective of this study was to describe the clinical features and the genetic cause of GT in a 6-year-old girl from south China. Methods A three-generation family was studied. The proband patient aged 6 years and her parents undertook examinations of platelet counts, blood film, bleeding time, platelet aggregation, and flow cytometry. All coding exons of the ITGA2B and ITGB3 genes were amplified by polymerase chain reaction （PCR）, and direct sequencing was performed for mutational screening on the patient and normal controls consisted of 52 healthy blood donors. Reverse transcription PCR was conducted to test for exon skipping. Results The proposita patient showed dispersing platelets, prolonged bleeding time, and severely reduced platelet aggregation in response to the physiological agonists adenosine diphosphate （ADP）, epinephrine, collagen, and ristocetin. Flow cytometric measurements showed that the contents of allb and 133 were significantly decreased. Sequencing results demonstrated two different types of heterozygous mutations existed in the allb gene （c.2930delG and IVS15-1delG）. The compound mutations were also confirmed in the patient＇s mother and father separately. Conclusions The allbβ3 deficiency of the proband was caused by two compound ITGA2B mutations, which were first reported in Chinese GT patients. The IVS15-1delG was first confirmed to cause an exon skipping.

关键词： Glanzmann thrombasthenia integrin alIIbβ mutation, exon skipping hemorrhage

Observation of acoustic hybrid topological phases induced by the p-d orbital interactions

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Science China(Physics,Mechanics & Astronomy) 2025年第1期Mechanics & Astronomy) .卷 132-139页

作者： Xiao Xiang Feng Gao Yu-Gui Peng Peng Wu Zong-lin Li Xue-Feng zhu School of Physics and Innovation Institute Huazhong University of Science and Technology

In spinless systems,growing attention has recently been attracted to synthetic gauge fields,which redefine the fundamental crystal symmetries by utilizing the projective algebraic ***,synthetic gauge fields have been commonly investigated in single orbital systems,and explorations on hybrid orbitals remain elusive in ***,we report the experimental observation of hybrid topological phases induced by hybrid synthetic gauge flux,which is formed by the interaction between p and d orbitals embedded in acoustic *** breaking the translation symmetries of Lxand/or Ly,we unambiguously demonstrate the first-order M?bius edge states and higher-order corner *** work establishes a platform for seeking exotic topological phases induced by hybrid-orbitals interactions and initializing the framework of hybrid-orbitalsrelated topological *** applications can be anticipated in scenarios such as high-quality sensing and robust wave trapping due to the stepwise energy distribution of the hybrid topological phase.

关键词： topological insulator synthetic gauge field projective symmetry corner states

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形状记忆聚氨酯在丝绸中应用的研究

丝绸 2006年第6期43卷 34-35,43页

作者：丁智超朱永林浙江理工大学材料与纺织学院杭州310018

利用涂层工艺,将不同浓度的形状记忆聚氨酯涂覆到丝绸中,制得含有一定量形状记忆聚氨酯的丝绸材料,并对其结构性能等进行了测试。结果表明,经形状记忆聚氨酯处理后的丝绸产品具有很好的形状记忆功能,成为形状记忆的丝绸产品。

关键词：丝织物形状记忆聚氨酯

Two Sphingolipids Isolated from a Mangrove Endophytic Fungus

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Two Sphingolipids Isolated from a Mangrove Endophytic Fungus

Non-coding RNAs in acute ischemic stroke:from brain to periphery

中国化学会第四届有机化学学术会议

作者： zhu, Feng lin, yong-Cheng (Department of Chemistry & Chemical Engineering, Foshan University, Foshan 528000) (School of Chemistry & Chemical Engineering, Zhongshan University, Guangzhou 510275)

＜正＞In the course of screening for novel structures from marine fungi, two sphingolipids were isolated firstly from the mycelium of a mangrove endophytic fungus （strain #1924） derived from the South China Sea. Their structures were elucidated as N-2’-hydroxypalmitoy1-1-O-β-D-glucopyranosyl-9-methy1-4E,8E-sphingadiene （A） and N-palmitoyldihydrosphingosine （B） by comprehensive spectra methods （mainly by 2D NMR）.

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Neural Regeneration Research 2025年第1期20卷 116-129页

作者： Shuo Li Zhaohan Xu Shiyao Zhang Huiling Sun Xiaodan Qin lin zhu Teng Jiang Junshan Zhou Fuling Yan Qiwen Deng Department of Neurology Nanjing First HospitalNanjing Medical UniversityNanjingJiangsu ProvinceChina Department of Neurology Zhongda HospitalSchool of MedicineSoutheast UniversityNanjingJiangsu ProvinceChina General Clinical Research Center Nanjing First HospitalNanjing Medical UniversityNanjingJiangsu ProvinceChina

Acute ischemic stroke is a clinical emergency and a condition with high morbidity,mortality,and *** predictive,diagnostic,and prognostic biomarkers and effective therapeutic targets for acute ischemic stroke remain *** innovations in high-throughput gene sequencing analysis,many aberrantly expressed non-coding RNAs(ncRNAs)in the brain and peripheral blood after acute ischemic stroke have been found in clinical samples and experimental *** expressed ncRNAs in the post-stroke brain were demonstrated to play vital roles in pathological processes,leading to neuroprotection or deterioration,thus ncRNAs can serve as therapeutic targets in acute ischemic ***,distinctly expressed ncRNAs in the peripheral blood can be used as biomarkers for acute ischemic stroke prediction,diagnosis,and *** particular,ncRNAs in peripheral immune cells were recently shown to be involved in the peripheral and brain immune response after acute ischemic *** this review,we consolidate the latest progress of research into the roles of ncRNAs(microRNAs,long ncRNAs,and circular RNAs)in the pathological processes of acute ischemic stroke–induced brain damage,as well as the potential of these ncRNAs to act as biomarkers for acute ischemic stroke prediction,diagnosis,and *** from this review will provide novel ideas for the clinical application of ncRNAs in acute ischemic stroke.

关键词： acute ischemic stroke apoptosis blood–brain barrier damage circular RNAs excitatory toxicity long non-coding RNAs microRNAs neuroinflammation non-coding RNAs oxidative stress

不同HSS评分的K—L各期膝关节炎患者采用关节镜下清理联合康复治疗的疗效评价

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医学临床研究 2016年第3期33卷 479-482页

作者：朱勇朱永林王效柱柳州市中医院骨三科广西柳州545001

【目的】探讨不同美国特种外科医院膝关节功能评分（HSS）及Kellgren—Lawrance（K—L）分级患者采用关节镜治疗及术后康复治疗的疗效差异，明确关节镜及康复治疗对膝关节骨性关节炎的治疗有效范围。【方法】回顾性分析2010年至2013年... 详细信息

【目的】探讨不同美国特种外科医院膝关节功能评分（HSS）及Kellgren—Lawrance（K—L）分级患者采用关节镜治疗及术后康复治疗的疗效差异，明确关节镜及康复治疗对膝关节骨性关节炎的治疗有效范围。【方法】回顾性分析2010年至2013年在本院实施关节镜清创及术后康复治疗的120例（126膝）患者的临床资料，根据膝关节HSS评分及K-L分级进行分组，对治疗前后不同分级标准下患者膝关节功能评分变化情况进行比较分析。【结果】术前HSS〉70分的K—L分级Ⅰ级、Ⅱ级、Ⅲ级患者的治疗后HSS评分较治疗前均显著的提高（P〈0．05），K—L分级Ⅰ级、Ⅱ级、Ⅲ级三组患者的HSS评分提高率差异无显著性（P〉0．05）。术前60-69分的K—L分级Ⅰ级、Ⅱ级、Ⅲ级患者的治疗后HSS评分较治疗前均显著的提高（P〈0．05），K—L分级Ⅰ级、Ⅱ级、Ⅲ级三组患者的HSS评分提高率差异无显著性（P〉0．05）。术前〈60分的K—L分级为Ⅱ级的患者的治疗后HSS评分较治疗前显著的提高（P〈0．05），K—L分级Ⅲ级、Ⅳ级患者治疗后HSS评分较治疗前差异无显著性（P〉0．05）；治疗后HSS评分提高率K—LⅡ级患者显著高于Ⅲ级、Ⅳ级（P〈0．05）。【结论】对于HSS评分〉60分或HSS评分〈60分且K—L分级Ⅱ级以上的患者采用关节镜清创及术后康复相结合治疗具有较好的疗效，但是对于HSS评分〈60分且K—L分级为Ⅲ级、Ⅳ级患者的治疗效果并不理想，可以考虑采用其他治疗方式。

关键词：骨关节炎,膝/治疗关节镜检查康复

龙血通络胶囊联合长春西汀治疗血栓性脑梗死恢复期的疗效观察

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现代药物与临床 2020年第3期35卷 469-472页

作者：朱永林郑州大学第二附属医院老年病三科河南郑州450014

目的分析龙血通络联合长春西汀注射液治疗血栓性脑梗死恢复期的临床效果。方法选择2017年7月-2019年2月郑州大学第二附属医院收治的143例血栓性脑梗死恢复期患者,随机分成对照组(71例)和治疗组(72例)。对照组静脉滴注长春西汀注射液,30... 详细信息

目的分析龙血通络联合长春西汀注射液治疗血栓性脑梗死恢复期的临床效果。方法选择2017年7月-2019年2月郑州大学第二附属医院收治的143例血栓性脑梗死恢复期患者,随机分成对照组(71例)和治疗组(72例)。对照组静脉滴注长春西汀注射液,30 mg加入生理盐水,1次/d。治疗组在对照组基础上口服龙血通络胶囊,2粒/次,3次/d。两组患者均治疗2周。观察两组患者临床疗效,同时比较治疗前后两组患者平均脑血流速度、三酰甘油(TG)、低密度脂蛋白(LDL)、高密度脂蛋白(HDL-C)和总胆固醇(TC)水平。结果治疗后,对照组临床有效率为76.06%,显著低于治疗组的93.06%,两组比较差异具有统计学意义(P<0.05)。治疗后,两组大脑中动脉(MCA)、大脑后动脉(PCA)、大脑前动脉(ACA)、基底动脉(BA)平均血流速度均显著增加(P<0.05),且治疗组平均血流速度明显高于对照组(P<0.05)。治疗后,两组TG、LDL和TG水平均得到明显降低(P<0.05),而HDL-C水平明显升高(P<0.05),且治疗组血脂水平均明显优于对照组(P<0.05)。结论龙血通络联合长春西汀注射液治疗脑血栓恢复期患者,可有效改善患者临床症状,促进脑部血液循环,优化神经功能和血脂水平,具有一定的临床推广应用价值。

关键词：龙血通络胶囊长春西汀注射液血栓性脑梗死恢复期脑血流速度三酰甘油低密度脂蛋白高密度脂蛋白