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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 potential role of Connexin 43 protein treatment i

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

Research and Exploration of Ideological and Political Education in the Course of pathophysiology

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Open Journal of Applied Sciences 2024年第5期14卷 1364-1379页

作者： Tongtong Lv Qiong Wang Yanhua Zhang Xiaochun Peng Department of pathophysiology School of Basic Medicine Health Science Center Yangtze University Jingzhou China Department of Nursing Health Science Center Yangtze University Jingzhou China Department of Physiology School of Basic Medicine Health Science Center Yangtze University Jingzhou China Laboratory of Oncology Center for Molecular Medicine School of Basic Medicine Health Science Center Yangtze University Jingzhou China

Course based ideological and political education (CIPE) is an important way to improve the quality of ideological and political work and talent cultivation. This study explores for the first time the implementation of ideological and political education in the teaching of pathophysiology courses, and also analyzes the evaluation of student psychological status and the importance of mental health education in the implementation of IPE courses. A survey was conducted on 211 students at Yangtze University to understand their motivation and behavior towards learning ideological, political, and pathophysiological courses. In addition, a questionnaire survey was used to explore the relationship between pathophysiology and ideological and political courses, as well as the importance of their satisfaction with the implementation of ideological and political courses in pathophysiology and mental health education. The research results indicate that factors such as educational background and gender differences affect the learning of CIPE. Graduate students are more interested in ideological and political courses, while female students find it difficult to study pathophysiology;In addition, the results of one-way ANOVA indicate that the implementation effect of IPE in pathophysiology courses depends on the level of interest in IPE and pathophysiology courses, the level of consideration for the importance of professional courses, the professional gains after studying pathophysiology, and the level of understanding of the relationship between IPE and CIPE. 81.04% of students believe that in the CIPE process, telling stories by teachers themselves is the most popular way of communication and education. This reflects the importance of mental health education from the perspective of CIPE. In addition, this study also indicates that PBL and flipped classroom teaching models are popular teaching models in CIPE. This study is beneficial for promoting the improvement and implementation of CIPE and mental health education in higher ed

关键词： pathophysiology Curriculum Ideological and Political Education Higher Education Psychology Questionnaire Survey

The advantages of multi-level omics research on stem cell-based therapies for ischemic stroke

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Neural Regeneration Research 2024年第9期19卷 1998-2003页

作者： Yiqing Wang Chuheng Chang Renzhi Wang Xiaoguang Li Xinjie Bao 4+4 Doctor Medical Program Chinese Academy of Medical Sciences&Peking Union Medical CollegeBeijingChina Department of Neurosurgery Peking Union Medical College HospitalPeking Union Medical CollegeChinese Academy of Medical SciencesBeijingChina Beijing Key Laboratory for Biomaterials and Neural Regeneration School of Biological Science and Medical EngineeringBeihang UniversityBeijingChina Department of Neurobiology School of Basic Medical SciencesCapital Medical UniversityBeijingChina

Stem cell transplantation is a potential therapeutic strategy for ischemic stroke. However, despite many years of preclinical research, the application of stem cells is still limited to the clinical trial stage. Although stem cell therapy can be highly beneficial in promoting functional recovery, the precise mechanisms of action that are responsible for this effect have yet to be fully elucidated. Omics analysis provides us with a new perspective to investigate the physiological mechanisms and multiple functions of stem cells in ischemic stroke. Transcriptomic, proteomic, and metabolomic analyses have become important tools for discovering biomarkers and analyzing molecular changes under pathological conditions. Omics analysis could help us to identify new pathways mediated by stem cells for the treatment of ischemic stroke via stem cell therapy, thereby facilitating the translation of stem cell therapies into clinical use. In this review, we summarize the pathophysiology of ischemic stroke and discuss recent progress in the development of stem cell therapies for the treatment of ischemic stroke by applying multi-level omics. We also discuss changes in RNAs, proteins, and metabolites in the cerebral tissues and body fluids under stroke conditions and following stem cell treatment, and summarize the regulatory factors that play a key role in stem cell therapy. The exploration of stem cell therapy at the molecular level will facilitate the clinical application of stem cells and provide new treatment possibilities for the complete recovery of neurological function in patients with ischemic stroke.

关键词： ischemic stroke mesenchymal stem cells metabolomics multilevel omics neural stem/progenitor cells neuroinflammation pathophysiology proteomics stem cell therapy transcriptomes

Pharmacotherapy of urethral stricture

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Asian Journal of Andrology 2024年第1期26卷 1-9页

作者： Hui Luo Ke-Cheng Lou Ling-Yu Xie Fei Zeng Jun-Rong Zou The First Clinical College Gannan Medical UniversityGanzhou 341000China Department of Urology The First Affiliated Hospital of Gannan Medical UniversityGanzhou 341000China Institute of Urology The First Affiliated Hospital of Gannan Medical UniversityGanzhou 341000China Jiangxi Engineering Technology Research Center of Calculi Prevention Ganzhou 341000China

Urethral stricture is characterized by the chronic formation of fibrous tissue,leading to the narrowing of the urethral lumen.Despite the availability of various endoscopic treatments,the recurrence of urethral strictures remains a common challenge.Postsurgery pharmacotherapy targeting tissue fibrosis is a promising option for reducing recurrence rates.Although drugs cannot replace surgery,they can be used as adjuvant therapies to improve outcomes.In this regard,many drugs have been proposed based on the mechanisms underlying the pathophysiology of urethral stricture.Ongoing studies have obtained substantial progress in treating urethral strictures,highlighting the potential for improved drug effectiveness through appropriate clinical delivery methods.Therefore,this review summarizes the latest researches on the mechanisms related to the pathophysiology of urethral stricture and the drugs to provide a theoretical basis and new insights for the effective use and future advancements in drug therapy for urethral stricture.

关键词： adjuvant therapy pathophysiology pharmacotherapy urethra urethral stricture

Understanding the spectrum of non-motor symptoms in multiple sclerosis:insights from animal models

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Neural Regeneration Research 2024年第1期19卷 84-91页

作者： Poornima D.E.Weerasinghe-Mudiyanselage Joong-Sun Kim Taekyun Shin Changjong Moon Department of Veterinary Anatomy and Animal Behavior College of Veterinary Medicine and BK21 FOUR programChonnam National UniversityGwangjuRepublic of Korea Department of Veterinary Anatomy College of Veterinary Medicine and Veterinary Medical Research InstituteJeju National UniversityJejuRepublic of Korea

Multiple sclerosis is a chronic autoimmune disease of the central nervous system and is generally considered to be a non-traumatic,physically debilitating neurological disorder.In addition to experiencing motor disability,patients with multiple sclerosis also experience a variety of nonmotor symptoms,including cognitive deficits,anxiety,depression,sensory impairments,and pain.However,the pathogenesis and treatment of such non-motor symptoms in multiple scle rosis are still under research.Preclinical studies for multiple sclerosis benefit from the use of disease-appropriate animal models,including experimental autoimmune encephalomyelitis.Prior to understanding the pathophysiology and developing treatments for non-motor symptoms,it is critical to chara cterize the animal model in terms of its ability to replicate certain non-motor features of multiple sclerosis.As such,no single animal model can mimic the entire spectrum of symptoms.This review focuses on the non-motor symptoms that have been investigated in animal models of multiple sclerosis as well as possible underlying mechanisms.Further,we highlighted gaps in the literature to explain the nonmotor aspects of multiple sclerosis in expe rimental animal models,which will serve as the basis for future studies.

关键词： anxiety cognitive deficit depression experimental autoimmune encephalomyelitis motor disability neurological disorder pain pathophysiology preclinical study sensory impairments

Traditional Chinese medicine for the treatment of immune-related nephropathy:A review

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Acta Pharmaceutica Sinica B 2024年第1期14卷 38-66页

作者： Pu Jiang Changliang Yao De-an Guo School of Pharmacy Nanjing University of Chinese MedicineNanjing 210023China Shanghai Research Center for Modernization of Traditional Chinese Medicine National Engineering Research Center of TCM Standardization TechnologyShanghai Institute of Materia MedicaChinese Academy of SciencesShanghai 201203China

Immune-related nephropathy(IRN)refers to immune-response-mediated glomerulonephritis and is the main cause of end-stage renal failure.The pathogenesis of IRN is not fully understood;therefore,treatment is challenging.Traditional Chinese medicines(TCMs)have potent clinical effects in the treatment of the IRN conditions immunoglobulin A nephropathy,lupus nephropathy,and diabetic nephropathy.The underlying mechanisms mainly include its inhibition of inflammation;improvements to renal interstitial fibrosis,oxidative stress,autophagy,apoptosis;and regulation of immunity.In this review,we summarize the clinical symptoms of the three IRN subtypes and the use of TCM prescriptions,herbs,and bioactive compounds in treating IRN,as well as the potential mechanisms,intending to provide a reference for the future study of TCM as IRN treatments.

关键词： IgA nephropathy Diabetic nephropathy Lupus nephritis Traditional Chinese medicine Immunity Pathogenesis Mechanism pathophysiology

Type 2 diabetes in children and adolescents:Exploring the disease heterogeneity and research gaps to optimum management

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World Journal of Clinical Pediatrics 2024年第2期13卷 80-99页

作者： Subhodip Pramanik Sunetra Mondal Rajan Palui Sayantan Ray Department of Endocrinology Neotia Getwel Multi-specialty hospitalSiliguri 734010West BengalIndia Department of Endocrinology NRS Medical College and HospitalKolkata 700014West BengalIndia Department of Endocrinology All India Institute of Medical SciencesBhubaneswarBhubaneswar 751019OdishaIndia

Over the past 20 years,the incidence and prevalence of type 2 diabetes mellitus(T2DM)in children and adolescents have increased,particularly in racial and ethnic minorities.Despite the rise in T2DM in children and adolescents,the pathophysiology and progression of disease in this population are not clearly understood.Youth-onset T2DM has a more adverse clinical course than is seen in those who develop T2DM in adulthood or those with T1DM.Furthermore,the available therapeutic options are more limited for children and adolescents with T2DM compared to adult patients,mostly due to the challenges of implementing clinical trials.A better understanding of the mechanisms underlying the development and aggressive disease phenotype of T2DM in youth is important to finding effective prevention and management strategies.This review highlights the key evidence about T2DM in children and adolescents and its current burden and challenges both in clinical care and research activities.

关键词： Type 2 diabetes mellitus Children and adolescents pathophysiology Heterogeneity Complications Treatment options Barriers

Hereditary severe insulin resistance syndrome:Pathogenesis,pathophysiology,and clinical management

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Genes & Diseases 2023年第5期10卷 1846-1856页

作者： Junaid Iqbal Hong-Li Jiang Hui-Xuan Wu Long Li Ying-Hui Zhou Nan Hu Fen Xiao Ting Wang Shi-Na Xu Hou-De Zhou National Clinical Research Center for Metabolic Diseases Hunan Provincial Key Laboratory for Metabolic Bone Diseasesand Department of Endocrinology and MetabolismThe Second XiangYa Hospital of Central South UniversityChangshaHunan 410011China

Severe insulin resistance has been linked to some of the most globally prevalent disorders,such as diabetes mellitus,nonalcoholic fatty liver disease,polycystic ovarian syndrome,and hypertension.Hereditary severe insulin resistance syndrome(H-SIRS)is a rare disorder classified into four principal categories:primary insulin receptor defects,lipodystrophies,complex syndromes,and obesity-related H-SIRS.Genes such as INSR,AKT2,TBC1D4,AGPAT2,BSCL2,CAV1,PTRF,LMNA,PPARG,PLIN1,CIDEC,LIPE,PCYT1A,MC4R,LEP,POMC,SH2B1,RECQL2,RECQL3,ALMS1,PCNT,ZMPSTE24,PIK3R1,and POLD1 have been linked to H-SIRS.Its clinical features include insulin resistance,hyperglycemia,hyperandrogenism,severe dyslipidemia,fatty liver,abnormal topography of adipose tissue,and low serum leptin and adiponectin levels.Diagnosis of H-SIRS is based on the presence of typical clinical features associated with the various H-SIRS forms and the identification of mutations in H-SIRS-linked genes by genetic testing.Diet therapy,insulin sensitization,exogenous insulin therapy,and leptin replacement therapy have widely been adopted to manage H-SIRS.The rarity of H-SIRS,its highly variable clinical presentation,refusal to be tested for genetic mutations by patients’family members who are not severely sick,unavailability of genetic testing,and testing expenses contribute to the delayed or underdiagnoses of H-SIRS.Early diagnosis facilitates early management of the condition,which results in improved glycemic control and delayed onset of diabetes and other complications related to severe insulin resistance.The use of updated genetic sequencing technologies is recommended,and long-term studies are required for genotype–phenotype differentiation and formulation of diagnostic and treatment protocols.

关键词： Diabetes Genetics Insulin resistance Lipodystrophy pathophysiology Therapy

Chinese Medicine Meets Conventional Medicine in Targeting COVID-19 pathophysiology,Complications and Comorbidities

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Chinese Journal of Integrative Medicine 2022年第7期28卷 627-635页

作者： WANG Shan-shan ZENG Xian WANG Ya-li Zhuoma Dongzhi ZHAO Yu-fen CHEN Yu-zong Qian Xuesen Collaborative Research Center of Astrochemistry and Space Life Sciences Institute of Drug Discovery TechnologyNingbo UniversityNingboZhejiang Province 315211China Department of Biological Medicines&Shanghai Engineering Research Center of Immunotherapeutics Fudan University School of PharmacyShanghai 201203China Bioinformatics and Drug Design Group Department of PharmacyNational University of Singapore117543Singapore Medical College Tibet UniversityLhasa 850011China Department of Chemical Biology College of Chemistry and Chemical Engineeringand The Key Laboratory for Chemical Biology of Fujian ProvinceXiamen UniversityXiamenFujian Province 361005China Key Laboratory of Bioorganic Phosphorus Chemistry and Chemical Biology(Ministry of Education) Department of ChemistryTsinghua UniversityBeijing 102206China

Objective: To investigate how the National Health Commission of China(NHCC)-recommended Chinese medicines(CMs) modulate the major maladjustments of coronavirus disease 2019(COVID-19),particularly the clinically observed complications and comorbidities. Methods: By focusing on the potent targets in common with the conventional medicines, we investigated the mechanisms of 11 NHCC-recommended CMs in the modulation of the major COVID-19 pathophysiology(hyperinflammations, viral replication), complications(pain, headache) and comorbidities(hypertension, obesity, diabetes). The constituent herbs of these CMs and their chemical ingredients were from the Traditional Chinese Medicine Information Database. The experimentally-determined targets and the activity values of the chemical ingredients of these CMs were from the Natural Product Activity and Species Source Database. The approved and clinical trial drugs against these targets were searched from the Therapeutic Target Database and DrugBank Database. Pathways of the targets was obtained from Kyoto Encyclopedia of Genes and Genomes and additional literature search. Results: Overall,9 CMs modulated 6 targets discovered by the COVID-19 target discovery studies, 8 and 11 CMs modulated 8and 6 targets of the approved or clinical trial drugs for the treatment of the major COVID-19 complications and comorbidities, respectively. Conclusion: The coordinated actions of each NHCC-recommended CM against a few targets of the major COVID-19 pathophysiology, complications and comorbidities, partly have common mechanisms with the conventional medicines.

关键词： COVID-19 Chinese medicine target therapeutic mechanism pathophysiology comorbidity