成骨不全的综合综述:目前的治疗方法和未来的创新。

IF 3.9 3区 医学 Q2 BIOTECHNOLOGY & APPLIED MICROBIOLOGY
Human gene therapy Pub Date : 2025-03-01 Epub Date: 2025-02-11 DOI:10.1089/hum.2024.191
Sachin Chaugule, Christodoulos Kypros Constantinou, Aijaz Ahmad John, Dimitra Micha, Marelise Eekhoff, Ellen Gravallese, Guangping Gao, Jae-Hyuck Shim
{"title":"成骨不全的综合综述:目前的治疗方法和未来的创新。","authors":"Sachin Chaugule, Christodoulos Kypros Constantinou, Aijaz Ahmad John, Dimitra Micha, Marelise Eekhoff, Ellen Gravallese, Guangping Gao, Jae-Hyuck Shim","doi":"10.1089/hum.2024.191","DOIUrl":null,"url":null,"abstract":"<p><p>Osteogenesis imperfecta (OI) is a rare genetic disorder characterized by bone fragility due to reduced bone quality, often accompanied by low bone mass, recurrent fractures, hearing loss, skeletal abnormalities, and short stature. Pathogenic variants in over 20 genes lead to clinical and genetic variability in OI, resulting in diverse symptoms and severity. Current management involves a multidisciplinary approach, including antiresorptive medications, physiotherapy, occupational therapy, and orthopedic surgery, which provide symptomatic relief but no cure. Advancements in gene therapy technologies and stem cell therapies offer promising prospects for long-lasting or permanent solutions. This review provides a comprehensive overview of OI's classification, pathogenesis, and current treatment options. It also explores emerging biotechnologies for stem cells and gene-targeted therapies in OI. The potential of these innovative therapies and their clinical implementation challenges are evaluated, focusing on their imminent success in treating bone disorders.</p>","PeriodicalId":13007,"journal":{"name":"Human gene therapy","volume":" ","pages":"597-617"},"PeriodicalIF":3.9000,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11971546/pdf/","citationCount":"0","resultStr":"{\"title\":\"Comprehensive Review of Osteogenesis Imperfecta: Current Treatments and Future Innovations.\",\"authors\":\"Sachin Chaugule, Christodoulos Kypros Constantinou, Aijaz Ahmad John, Dimitra Micha, Marelise Eekhoff, Ellen Gravallese, Guangping Gao, Jae-Hyuck Shim\",\"doi\":\"10.1089/hum.2024.191\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Osteogenesis imperfecta (OI) is a rare genetic disorder characterized by bone fragility due to reduced bone quality, often accompanied by low bone mass, recurrent fractures, hearing loss, skeletal abnormalities, and short stature. Pathogenic variants in over 20 genes lead to clinical and genetic variability in OI, resulting in diverse symptoms and severity. Current management involves a multidisciplinary approach, including antiresorptive medications, physiotherapy, occupational therapy, and orthopedic surgery, which provide symptomatic relief but no cure. Advancements in gene therapy technologies and stem cell therapies offer promising prospects for long-lasting or permanent solutions. This review provides a comprehensive overview of OI's classification, pathogenesis, and current treatment options. It also explores emerging biotechnologies for stem cells and gene-targeted therapies in OI. The potential of these innovative therapies and their clinical implementation challenges are evaluated, focusing on their imminent success in treating bone disorders.</p>\",\"PeriodicalId\":13007,\"journal\":{\"name\":\"Human gene therapy\",\"volume\":\" \",\"pages\":\"597-617\"},\"PeriodicalIF\":3.9000,\"publicationDate\":\"2025-03-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11971546/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Human gene therapy\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1089/hum.2024.191\",\"RegionNum\":3,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2025/2/11 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q2\",\"JCRName\":\"BIOTECHNOLOGY & APPLIED MICROBIOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Human gene therapy","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1089/hum.2024.191","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/2/11 0:00:00","PubModel":"Epub","JCR":"Q2","JCRName":"BIOTECHNOLOGY & APPLIED MICROBIOLOGY","Score":null,"Total":0}
引用次数: 0

摘要

成骨不全症(Osteogenesis imperfecta, OI)是一种罕见的遗传性疾病,其特征是由于骨质量降低而导致骨脆性,通常伴有低骨量、复发性骨折、听力丧失、骨骼异常和身材矮小。20多个基因的致病变异导致成骨不全的临床和遗传变异,导致不同的症状和严重程度。目前的治疗涉及多学科的方法,包括抗吸收药物、物理治疗、职业治疗和骨科手术,这些方法可以缓解症状,但不能治愈。基因治疗技术和干细胞治疗的进步为长期或永久的解决方案提供了良好的前景。本文综述了成骨不全症的分类、发病机制和目前的治疗方案。它还探讨了成骨不全症中干细胞和基因靶向治疗的新兴生物技术。评估了这些创新疗法的潜力及其临床实施挑战,重点是它们在治疗骨骼疾病方面即将取得的成功。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Comprehensive Review of Osteogenesis Imperfecta: Current Treatments and Future Innovations.

Osteogenesis imperfecta (OI) is a rare genetic disorder characterized by bone fragility due to reduced bone quality, often accompanied by low bone mass, recurrent fractures, hearing loss, skeletal abnormalities, and short stature. Pathogenic variants in over 20 genes lead to clinical and genetic variability in OI, resulting in diverse symptoms and severity. Current management involves a multidisciplinary approach, including antiresorptive medications, physiotherapy, occupational therapy, and orthopedic surgery, which provide symptomatic relief but no cure. Advancements in gene therapy technologies and stem cell therapies offer promising prospects for long-lasting or permanent solutions. This review provides a comprehensive overview of OI's classification, pathogenesis, and current treatment options. It also explores emerging biotechnologies for stem cells and gene-targeted therapies in OI. The potential of these innovative therapies and their clinical implementation challenges are evaluated, focusing on their imminent success in treating bone disorders.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
Human gene therapy
Human gene therapy 医学-生物工程与应用微生物
CiteScore
6.50
自引率
4.80%
发文量
131
审稿时长
4-8 weeks
期刊介绍: Human Gene Therapy is the premier, multidisciplinary journal covering all aspects of gene therapy. The Journal publishes in-depth coverage of DNA, RNA, and cell therapies by delivering the latest breakthroughs in research and technologies. Human Gene Therapy provides a central forum for scientific and clinical information, including ethical, legal, regulatory, social, and commercial issues, which enables the advancement and progress of therapeutic procedures leading to improved patient outcomes, and ultimately, to curing diseases.
×
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×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
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