争论-何时以及如何使用依地苯酮治疗LHON?

IF 2.8 3区 医学 Q1 OPHTHALMOLOGY
Marcela Votruba
{"title":"争论-何时以及如何使用依地苯酮治疗LHON?","authors":"Marcela Votruba","doi":"10.1111/aos.16902","DOIUrl":null,"url":null,"abstract":"<div>\n \n <section>\n \n <p>The International Consensus Statement on the Clinical and Therapeutic Management of Leber Hereditary Optic Neuropathy was published in 2017 based on data available in 2016. The intent was to provide expert consensus statements for the clinical and therapeutic management of LHON based on the currently available evidence. It provided the guidelines for clinical and therapeutic management of LHON. It established a number of benchmarks such as clinical staging of LHION into Asymptomatic (mutation carriers), Subacute (&lt;6 months from onset), Dynamic (6–12 months) and Chronic (&gt;12 months) stages. We can all agree on the criteria for the diagnosis of LHON, based on a careful history, evaluation of key structural and functional visual parameters, and on a molecular confirmation of a pathogenic mtDNA mutation. On management it is clearly highly important to include genetic counselling and informing the patient about potentially preventable lifestyle risk factors. The use of idebenone is more debated and much has changed. Further studies and case series have been published and issues around when to start, how long to treat for and whether to treat chronic patients have all emerged. In this debate we shall raise a number of these issues and provide a reflection of the available data, their validity and clinical relevance and debate next steps.</p>\n </section>\n </div>","PeriodicalId":6915,"journal":{"name":"Acta Ophthalmologica","volume":"103 S284","pages":""},"PeriodicalIF":2.8000,"publicationDate":"2025-01-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/10.1111/aos.16902","citationCount":"0","resultStr":"{\"title\":\"The debate – when and how should i use idebenone for LHON?\",\"authors\":\"Marcela Votruba\",\"doi\":\"10.1111/aos.16902\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div>\\n \\n <section>\\n \\n <p>The International Consensus Statement on the Clinical and Therapeutic Management of Leber Hereditary Optic Neuropathy was published in 2017 based on data available in 2016. The intent was to provide expert consensus statements for the clinical and therapeutic management of LHON based on the currently available evidence. It provided the guidelines for clinical and therapeutic management of LHON. It established a number of benchmarks such as clinical staging of LHION into Asymptomatic (mutation carriers), Subacute (&lt;6 months from onset), Dynamic (6–12 months) and Chronic (&gt;12 months) stages. We can all agree on the criteria for the diagnosis of LHON, based on a careful history, evaluation of key structural and functional visual parameters, and on a molecular confirmation of a pathogenic mtDNA mutation. On management it is clearly highly important to include genetic counselling and informing the patient about potentially preventable lifestyle risk factors. The use of idebenone is more debated and much has changed. Further studies and case series have been published and issues around when to start, how long to treat for and whether to treat chronic patients have all emerged. In this debate we shall raise a number of these issues and provide a reflection of the available data, their validity and clinical relevance and debate next steps.</p>\\n </section>\\n </div>\",\"PeriodicalId\":6915,\"journal\":{\"name\":\"Acta Ophthalmologica\",\"volume\":\"103 S284\",\"pages\":\"\"},\"PeriodicalIF\":2.8000,\"publicationDate\":\"2025-01-19\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://onlinelibrary.wiley.com/doi/10.1111/aos.16902\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Acta Ophthalmologica\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://onlinelibrary.wiley.com/doi/10.1111/aos.16902\",\"RegionNum\":3,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"OPHTHALMOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Acta Ophthalmologica","FirstCategoryId":"3","ListUrlMain":"https://onlinelibrary.wiley.com/doi/10.1111/aos.16902","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"OPHTHALMOLOGY","Score":null,"Total":0}
引用次数: 0

摘要

《Leber遗传性视神经病变临床和治疗管理国际共识声明》基于2016年的现有数据于2017年发布。目的是根据现有证据,为LHON的临床和治疗管理提供专家共识声明。为LHON的临床和治疗提供指导。它建立了许多基准,如LHION的临床分期为无症状(突变携带者)、亚急性(发病后6个月)、动态(6 - 12个月)和慢性(12个月)阶段。基于仔细的病史、对关键结构和功能视觉参数的评估,以及对致病性mtDNA突变的分子确认,我们都可以就LHON的诊断标准达成一致。在管理方面,包括遗传咨询和告知患者潜在的可预防的生活方式风险因素显然非常重要。伊地苯酮的使用争议更大,而且已经发生了很大的变化。进一步的研究和病例系列已经发表,关于何时开始、治疗多长时间以及是否治疗慢性患者的问题都已经出现。在这次辩论中,我们将提出一些这样的问题,并提供对现有数据的反映,它们的有效性和临床相关性,并讨论下一步。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
The debate – when and how should i use idebenone for LHON?

The International Consensus Statement on the Clinical and Therapeutic Management of Leber Hereditary Optic Neuropathy was published in 2017 based on data available in 2016. The intent was to provide expert consensus statements for the clinical and therapeutic management of LHON based on the currently available evidence. It provided the guidelines for clinical and therapeutic management of LHON. It established a number of benchmarks such as clinical staging of LHION into Asymptomatic (mutation carriers), Subacute (<6 months from onset), Dynamic (6–12 months) and Chronic (>12 months) stages. We can all agree on the criteria for the diagnosis of LHON, based on a careful history, evaluation of key structural and functional visual parameters, and on a molecular confirmation of a pathogenic mtDNA mutation. On management it is clearly highly important to include genetic counselling and informing the patient about potentially preventable lifestyle risk factors. The use of idebenone is more debated and much has changed. Further studies and case series have been published and issues around when to start, how long to treat for and whether to treat chronic patients have all emerged. In this debate we shall raise a number of these issues and provide a reflection of the available data, their validity and clinical relevance and debate next steps.

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
Acta Ophthalmologica
Acta Ophthalmologica 医学-眼科学
CiteScore
7.60
自引率
5.90%
发文量
433
审稿时长
6 months
期刊介绍: Acta Ophthalmologica is published on behalf of the Acta Ophthalmologica Scandinavica Foundation and is the official scientific publication of the following societies: The Danish Ophthalmological Society, The Finnish Ophthalmological Society, The Icelandic Ophthalmological Society, The Norwegian Ophthalmological Society and The Swedish Ophthalmological Society, and also the European Association for Vision and Eye Research (EVER). Acta Ophthalmologica publishes clinical and experimental original articles, reviews, editorials, educational photo essays (Diagnosis and Therapy in Ophthalmology), case reports and case series, letters to the editor and doctoral theses.
×
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