Haemophilia最新文献

筛选
英文 中文
Interleukin-10 Mitigates Chondrocyte Ferroptosis Associated With Haemophilic Arthropathy via Modulation of the STAT3 Signalling Pathway 白细胞介素-10通过调节STAT3信号通路减轻与血友病关节病相关的软骨细胞下垂。
IF 3 2区 医学
Haemophilia Pub Date : 2025-08-25 DOI: 10.1111/hae.70093
Fudong Luo, Yi Hu, Yi Wang, Dasheng Luo, Tao Chen, Defu Yu
{"title":"Interleukin-10 Mitigates Chondrocyte Ferroptosis Associated With Haemophilic Arthropathy via Modulation of the STAT3 Signalling Pathway","authors":"Fudong Luo,&nbsp;Yi Hu,&nbsp;Yi Wang,&nbsp;Dasheng Luo,&nbsp;Tao Chen,&nbsp;Defu Yu","doi":"10.1111/hae.70093","DOIUrl":"10.1111/hae.70093","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Haemophilic arthropathy (HA) is characterized by recurrent intra-articular bleeding leading to cartilage degeneration. Ferroptosis plays a critical role in this process. While IL-10 is known to inhibit apoptosis, its effect on ferroptosis remains unexplored.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Aim</h3>\u0000 \u0000 <p>To investigate IL-10's role in chondrocyte ferroptosis in homophilic arthropathy via STAT3 signalling.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Cartilage samples from HA and OA patients were analyzed by TEM and Western blot for iron deposition and IL-10. ATDC5 chondrocytes were treated with FAC (iron overload), Erastin (ferroptosis inducer) and IL-10±STAT3 inhibitor (AG-490). Cell viability assays, Western blotting, reactive oxygen species detection and immunofluorescent staining were performed. Viability, ROS and pathway proteins were assessed.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>HA chondrocytes exhibited elevated mitochondrial iron deposition and reduced IL-10 versus OA controls. IL-10 suppressed ferroptosis with efficacy comparable to Fer-1, mechanistically dependent on STAT3 activation.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>IL-10 suppresses ferroptosis in HA chondrocytes primarily via STAT3 signalling, providing theoretical support for its therapeutic potential for HA.</p>\u0000 </section>\u0000 </div>","PeriodicalId":12819,"journal":{"name":"Haemophilia","volume":"31 5","pages":"1077-1085"},"PeriodicalIF":3.0,"publicationDate":"2025-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144951678","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Gap Analysis Investigating Healthcare Professionals’ Skills and Knowledge for Pain Management in Haemophilia—A Qualitative Study 血友病患者疼痛管理技能与知识的差距分析——质性研究。
IF 3 2区 医学
Haemophilia Pub Date : 2025-08-25 DOI: 10.1111/hae.70112
Paul McLaughlin, Pratima Chowdary, Catherine Harrison, David Stephensen, Nathalie Roussel
{"title":"Gap Analysis Investigating Healthcare Professionals’ Skills and Knowledge for Pain Management in Haemophilia—A Qualitative Study","authors":"Paul McLaughlin,&nbsp;Pratima Chowdary,&nbsp;Catherine Harrison,&nbsp;David Stephensen,&nbsp;Nathalie Roussel","doi":"10.1111/hae.70112","DOIUrl":"10.1111/hae.70112","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>People with haemophilia continue to experience pain impacting activities of daily living despite the advances in haemostatic treatments. Although guidelines exist, pain management in the clinical setting remains inadequate. It is unclear why this gap in practice persists. This study aimed to explore the practice and experience of pain management by haemophilia healthcare professionals (HCPs) to better understand perceived competency and barriers/facilitators to appropriate care.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>European HCPs working ≥ 3 years in haemophilia care were invited to participate (haematologists, nurses, physiotherapists and psychosocial professionals). Data were collected using profession-specific focus groups and semi-structured individual interviews, recorded and transcribed. The data were analysed using reflexive thematic analysis.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Twenty-three HCPs (6 haematologists, 7 nurses, 6 physiotherapists, 4 psychosocial professionals) from 10 European countries agreed to participate. Four themes were constructed: (1) Defining who is responsible: the challenges in multidisciplinary team provision for good pain management; (2) the concept of pain and its management exists in multiple temporalities; (3) aspirations for better pain management are limited by prevailing biomedicalism; (4) pain management can be better—knowledge and experience improves confidence.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Discussion</h3>\u0000 \u0000 <p>These data demonstrate a gap in confidence in the delivery of effective pain management, influenced by individual knowledge and skills and wider geographical determinants of healthcare delivery. There is a need for services to establish who is responsible for pain management and better understand professional role identity. Mechanisms to support the implementation of better pain care in haemophilia need to be explored.</p>\u0000 </section>\u0000 </div>","PeriodicalId":12819,"journal":{"name":"Haemophilia","volume":"31 5","pages":"988-996"},"PeriodicalIF":3.0,"publicationDate":"2025-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144951667","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Commentary: The Infected Blood Inquiry in the United Kingdom—Let the Dust Settle 评论:英国的受感染血液调查——让尘埃落定吧。
IF 3 2区 医学
Haemophilia Pub Date : 2025-08-25 DOI: 10.1111/hae.70116
Albert Farrugia
{"title":"Commentary: The Infected Blood Inquiry in the United Kingdom—Let the Dust Settle","authors":"Albert Farrugia","doi":"10.1111/hae.70116","DOIUrl":"10.1111/hae.70116","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <p>This commentary addresses issues raised during the infected blood inquiry (IBI) which ran for 6 years before concluding in 2024. Reference is made to publications in issues of Haemophilia and other journals, addressing the outcomes of the IBI from various perspectives. The author draws on his personal experience, as a patient who also made a career as a plasma fractionation scientist, to critique some of the IBI's salient conclusions. The commentary seeks to let the dust settle around the IBI by including features which have been inadequately addressed, in the author's view as a participant in the events covered by the inquiry.</p>\u0000 </section>\u0000 </div>","PeriodicalId":12819,"journal":{"name":"Haemophilia","volume":"31 5","pages":"811-814"},"PeriodicalIF":3.0,"publicationDate":"2025-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/hae.70116","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144951623","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Addressing Health Equity, Diversity, and Inclusion in the Bleeding Disorders Community: Insights From a Pilot Study of Multi-Stakeholder Interviews and Survey Data 解决出血性疾病社区的健康公平、多样性和包容性:来自多方利益相关者访谈和调查数据的试点研究的见解。
IF 3 2区 医学
Haemophilia Pub Date : 2025-08-25 DOI: 10.1111/hae.70118
Neil Frick, Keri L. Norris, Michael Pangrace, Terry Richardson, Sarah Waite-Ardini
{"title":"Addressing Health Equity, Diversity, and Inclusion in the Bleeding Disorders Community: Insights From a Pilot Study of Multi-Stakeholder Interviews and Survey Data","authors":"Neil Frick,&nbsp;Keri L. Norris,&nbsp;Michael Pangrace,&nbsp;Terry Richardson,&nbsp;Sarah Waite-Ardini","doi":"10.1111/hae.70118","DOIUrl":"10.1111/hae.70118","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Social determinants of health (SDOH) increasingly shape health care priorities, highlighting the intricate interplay of environment, behaviour, socioeconomics and health care access. The COVID-19 pandemic intensified disparities, particularly affecting racial/ethnic groups with bleeding disorders.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Aim</h3>\u0000 \u0000 <p>The pilot study aimed to identify key themes related to health equity, diversity, and inclusion as it relates to Black, indigenous people of colour (for example, Native Americans and Asian Pacific Islanders), and Hispanic/Latino people with bleeding disorders (PWBD) by interviewing haemophilia treatment centre (HTC) healthcare providers (HCPs) and a National Bleeding Disorders Foundation (NBDF) chapter executive director. It sought to identify practice gaps, understand the impact of SDOH, and provide an opportunity to propose interventions to address these disparities.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>NBDF conducted eight interviews with HTC staff members and an NBDF chapter executive director to explore the impact of bleeding disorders on underserved groups. Themes included barriers to care, SDOH influences, and potential interventions. A total of 33 HTC HCPs responded to an anonymous quantitative survey fielded electronically to further validate insights and gather quantitative data.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Specialists highlighted pandemic-related challenges, including exacerbated mental health concerns, communication barriers due to language, and transportation issues. These findings underscore the significant impact of SDOH on health care access and outcomes amongst underserved PWBD.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>The pilot study highlights the need to address access and health care disparities amongst underserved PWBD, particularly in racial/ethnic minorities. By addressing SDOH factors, cultural context, and healthcare professionals' bias, HTCs can strive towards equality, equity, and justice, facilitating optimal outcomes for all individuals with bleeding disorders.</p>\u0000 </section>\u0000 </div>","PeriodicalId":12819,"journal":{"name":"Haemophilia","volume":"31 5","pages":"997-1002"},"PeriodicalIF":3.0,"publicationDate":"2025-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144951661","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Safety and Efficacy of Damoctocog Alfa Pegol Prophylaxis in Patients With Severe Haemophilia A: Results of an Interventional, Post-Marketing Study. Damoctocog Alfa Pegol预防严重血友病A患者的安全性和有效性:一项介入性上市后研究的结果
IF 3 2区 医学
Haemophilia Pub Date : 2025-08-21 DOI: 10.1111/hae.70099
Pål André Holme, Lone Hvitfeldt Poulsen, Claudia Tueckmantel, Monika Maas Enriquez, María Teresa Alvarez Román, Raimondo De Cristofaro
{"title":"Safety and Efficacy of Damoctocog Alfa Pegol Prophylaxis in Patients With Severe Haemophilia A: Results of an Interventional, Post-Marketing Study.","authors":"Pål André Holme, Lone Hvitfeldt Poulsen, Claudia Tueckmantel, Monika Maas Enriquez, María Teresa Alvarez Román, Raimondo De Cristofaro","doi":"10.1111/hae.70099","DOIUrl":"https://doi.org/10.1111/hae.70099","url":null,"abstract":"<p><p> : This is a plain language summary of the results of a trial of damoctocog alfa pegol (BAY 94-9027, Jivi).</p>","PeriodicalId":12819,"journal":{"name":"Haemophilia","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144951609","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Dental Management for People With Congenital Haemophilia: A Systematic Review of the Existing Dental Recommendations 先天性血友病患者的牙科治疗:现有牙科建议的系统回顾。
IF 3 2区 医学
Haemophilia Pub Date : 2025-08-21 DOI: 10.1111/hae.70104
Mathangi Kumar, Sulochana Badagabettu, Keerthilatha M. Pai, Judith Angelitta Noronha, Baby S. Nayak, Penny McCarthy
{"title":"Dental Management for People With Congenital Haemophilia: A Systematic Review of the Existing Dental Recommendations","authors":"Mathangi Kumar,&nbsp;Sulochana Badagabettu,&nbsp;Keerthilatha M. Pai,&nbsp;Judith Angelitta Noronha,&nbsp;Baby S. Nayak,&nbsp;Penny McCarthy","doi":"10.1111/hae.70104","DOIUrl":"10.1111/hae.70104","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Haemophilia is the most common inherited bleeding disorder. Dental management for people with haemophilia (PwH) (both A and B) is challenging, as dental procedures may initiate bleeding episodes that may be difficult to control.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>This systematic review aimed to evaluate and summarize the existing dental guidelines that are proposed from various agencies/professional bodies, and so forth, for dental treatment procedures in PwH.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>A systematic electronic search on databases like Scopus, Medline, EMBASE, CINAHL, ProQuest and Web of Science was performed. The parameters evaluated were (a) clotting factor replacement therapy employed, (b) haemostatic agents, both local and systemic therapy and (c) dental procedural and pharmacological considerations that are recommended before, during, and after dental treatment procedures for people with mild, moderate and severe haemophilia. The dental treatment procedures included all minor and major procedures, including surgical extraction of wisdom teeth.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>A total of 11 guidelines were included for data extraction. The results indicated that all guidelines emphasized the importance of close collaboration among haematologists and dental professionals to ensure safe and effective dental treatment as a key component of comprehensive dental care. The various dental treatments (restorative, periodontal, orthodontic and prosthodontic treatments) and preventive aspects of oral health are detailed. Also, the amount of clotting factor replacement therapy for dental procedures, and pharmacological considerations recommended from the various professional organizations are discussed.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Well-designed large-scale research initiatives in dental management for PwH are necessary to understand the applicability and outcomes of such guidelines in clinical practice.</p>\u0000 \u0000 <p><b>Trial Registration</b>: REF/2024/01/077096</p>\u0000 </section>\u0000 </div>","PeriodicalId":12819,"journal":{"name":"Haemophilia","volume":"31 5","pages":"830-839"},"PeriodicalIF":3.0,"publicationDate":"2025-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/hae.70104","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144951655","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Acupuncture for Chronic Pain Management in Haemophilic Arthropathy: A Systematic Review 针刺治疗血友病关节病慢性疼痛:系统综述。
IF 3 2区 医学
Haemophilia Pub Date : 2025-08-21 DOI: 10.1111/hae.70109
Andrea Demeco, Marco Gusai, Maria Francesca Masi, Antonio Frizziero, Cosimo Costantino
{"title":"Acupuncture for Chronic Pain Management in Haemophilic Arthropathy: A Systematic Review","authors":"Andrea Demeco,&nbsp;Marco Gusai,&nbsp;Maria Francesca Masi,&nbsp;Antonio Frizziero,&nbsp;Cosimo Costantino","doi":"10.1111/hae.70109","DOIUrl":"10.1111/hae.70109","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Haemophilia is a severe hereditary bleeding disorder affecting ∼1 in 5000 males, caused by deficiencies in coagulation factors VIII or IX. Chronic pain from haemophilic arthropathy (HA), especially in target joints, reduces quality of life (HRQOL), range of motion (ROM) and daily function. While conventional treatment includes replacement therapy and rehabilitation, acupuncture has emerged as a complementary approach with potential pain-relief benefits and fewer side effects.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Aim</h3>\u0000 \u0000 <p>To evaluate the effectiveness of acupuncture in reducing chronic pain and improving function and quality of life in people with haemophilia (PWH).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Materials and Methods</h3>\u0000 \u0000 <p>This systematic review followed PRISMA guidelines (PROSPERO: CRD42024567714). Databases searched: PubMed, Scopus, Web of Science. PICO framework: (P) haemophilic patients, (I) acupuncture, (O) pain management. Studies on other coagulation disorders or non-acupuncture therapies were excluded. Methodological quality was assessed using the JBI score.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Of 514 records (464 PubMed, 40 Scopus, 10 Web of Science), 26 duplicates were removed, and 350 titles were screened. Seven met the inclusion criteria; after quality assessment and availability check, four studies were included. A total of 37 patients (mean age 41.4 years) reported meaningful pain reduction (VAS), reduced analgesic use and improved HRQOL. No significant bleeding events were reported.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Acupuncture may provide effective pain relief for haemophilic arthropathy with minimal side effects. However, larger, high-quality studies are needed to confirm its clinical benefits.</p>\u0000 </section>\u0000 </div>","PeriodicalId":12819,"journal":{"name":"Haemophilia","volume":"31 5","pages":"865-873"},"PeriodicalIF":3.0,"publicationDate":"2025-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/hae.70109","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144951591","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Development of Awareness Materials to Promote Early Diagnosis of Undiagnosed von Willebrand Disease: Integration of Multidisciplinary Perspectives Based on a Literature Review. 促进未确诊血管性血友病早期诊断的宣传材料的开发:基于文献综述的多学科视角整合。
IF 3 2区 医学
Haemophilia Pub Date : 2025-08-08 DOI: 10.1111/hae.70080
Azusa Nagao, Masahiro Takeyama, Kenichi Ogiwara, Akihiro Sawada, Yojiro Maruyama, Ryosuke Matsuno, Emika Kobayashi, Nana Ishida, Satoshi Higasa, Kagehiro Amano, Keiji Nogami, Yasuharu Nishida
{"title":"Development of Awareness Materials to Promote Early Diagnosis of Undiagnosed von Willebrand Disease: Integration of Multidisciplinary Perspectives Based on a Literature Review.","authors":"Azusa Nagao, Masahiro Takeyama, Kenichi Ogiwara, Akihiro Sawada, Yojiro Maruyama, Ryosuke Matsuno, Emika Kobayashi, Nana Ishida, Satoshi Higasa, Kagehiro Amano, Keiji Nogami, Yasuharu Nishida","doi":"10.1111/hae.70080","DOIUrl":"https://doi.org/10.1111/hae.70080","url":null,"abstract":"","PeriodicalId":12819,"journal":{"name":"Haemophilia","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144798928","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Identification and Analysis of Mitochondria-Related Proteins in Haemophilic Arthritis Synovial Membranes Based on Proteomic Analysis 基于蛋白质组学分析的血友病关节炎滑膜线粒体相关蛋白的鉴定和分析。
IF 3 2区 医学
Haemophilia Pub Date : 2025-08-08 DOI: 10.1111/hae.70110
Haitao Ma, Jiaxiang Zhang, Yongjie Yang, Liyin Liu, Defu Yu, Dasheng Luo, Tao Chen
{"title":"Identification and Analysis of Mitochondria-Related Proteins in Haemophilic Arthritis Synovial Membranes Based on Proteomic Analysis","authors":"Haitao Ma,&nbsp;Jiaxiang Zhang,&nbsp;Yongjie Yang,&nbsp;Liyin Liu,&nbsp;Defu Yu,&nbsp;Dasheng Luo,&nbsp;Tao Chen","doi":"10.1111/hae.70110","DOIUrl":"10.1111/hae.70110","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Haemophilic arthritis (HA), a severe complication in haemophiliacs, is driven by recurrent joint bleeding and iron overload from haemosiderin deposition, leading to synovial damage and joint dysfunction. Previous studies have examined the effects of iron overload on mitochondrial function, but the mechanisms linking iron metabolism disorders to mitochondrial dysfunction in HA remain poorly understood.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Comparative analyses of synovial samples from patients with advanced HA and osteoarthritis (OA) were performed using a proteomic approach. Differentially Expressed Proteins (DEPs) linked to mitochondrial function were identified and analysed for gene ontology (GO) and KEGG enrichment. A protein-protein interaction (PPI) network was constructed and findings were validated using Western blotting and Immunohistochemistry (IHC).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>A total of 152 mitochondria-associated DEPs were identified, significantly enriched in processes such as oxidative phosphorylation, aerobic respiration and the TCA cycle. KEGG analysis revealed disruption of pathways including oxidative phosphorylation and fatty acid metabolism, highlighting metabolic dysregulation in advanced HA synovium. TEM analysis revealed mitochondrial abnormalities including swelling, cristae breaks and iron deposition. NDUFS1 and SOD2 were significantly down-regulated in advanced HA synovium, linked to reduce mitochondrial efficiency and enhanced oxidative stress.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>This study identifies molecular pathways and proteins linked to mitochondrial dysfunction in advanced HA, including those involved in the TCA cycle, oxidative phosphorylation and oxidative stress responses. The reduction in NDUFS1 and SOD2 expression may be correlated with mitochondrial dysfunction caused by iron overload in synovial fibroblasts. They provide new insights into the pathogenesis of late-stage HA and offer potential avenues for mitochondria-targeted therapeutic strategies.</p>\u0000 </section>\u0000 </div>","PeriodicalId":12819,"journal":{"name":"Haemophilia","volume":"31 5","pages":"1066-1076"},"PeriodicalIF":3.0,"publicationDate":"2025-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144798929","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Utilising Thrombin Generation Assay to Guide Co-Administration of Factor Therapies With Fitusiran. 利用凝血酶生成试验指导因子治疗与非图西兰联合用药。
IF 3 2区 医学
Haemophilia Pub Date : 2025-08-08 DOI: 10.1111/hae.70108
Yesim Dargaud, Christophe Nougier, Stephanie Desage, Hamdi Rezigue, Amy Dericquebourg, Yohann Jourdy, Alexandre Leuci, Anne Lienhart
{"title":"Utilising Thrombin Generation Assay to Guide Co-Administration of Factor Therapies With Fitusiran.","authors":"Yesim Dargaud, Christophe Nougier, Stephanie Desage, Hamdi Rezigue, Amy Dericquebourg, Yohann Jourdy, Alexandre Leuci, Anne Lienhart","doi":"10.1111/hae.70108","DOIUrl":"https://doi.org/10.1111/hae.70108","url":null,"abstract":"","PeriodicalId":12819,"journal":{"name":"Haemophilia","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144798931","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
0
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
相关产品
×
本文献相关产品
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:604180095
Book学术官方微信