安德森-法布里病:当前诊断、心律失常风险分层和治疗策略综述。

IF 3.3 3区 医学 Q1 MEDICINE, GENERAL & INTERNAL Diagnostics Pub Date : 2025-01-09 DOI:10.3390/diagnostics15020139
Chiara Tognola, Giacomo Ruzzenenti, Alessandro Maloberti, Marisa Varrenti, Patrizio Mazzone, Cristina Giannattasio, Fabrizio Guarracini
{"title":"安德森-法布里病:当前诊断、心律失常风险分层和治疗策略综述。","authors":"Chiara Tognola, Giacomo Ruzzenenti, Alessandro Maloberti, Marisa Varrenti, Patrizio Mazzone, Cristina Giannattasio, Fabrizio Guarracini","doi":"10.3390/diagnostics15020139","DOIUrl":null,"url":null,"abstract":"<p><p>Anderson-Fabry disease (AFD) is a rare X-linked lysosomal storage disorder characterized by the accumulation of globotriaosylceramide, leading to multi-organ involvement and significant morbidity. Cardiovascular manifestations, particularly arrhythmias, are common and pose a considerable risk to affected individuals. This overview examines current approaches to arrhythmic risk stratification in AFD, focusing on the identification, assessment, and management of cardiac arrhythmias associated with the disease. We explore advancements in diagnostic techniques, including echocardiography, cardiac MRI, and ambulatory ECG monitoring, to enhance the detection of arrhythmogenic substrate. Furthermore, we discuss the role of genetic and biochemical markers in predicting arrhythmic risk and the implications for personalized treatment strategies. Current therapeutic interventions, including enzyme replacement therapy and antiarrhythmic medications, are reviewed in the context of their efficacy and limitations. Finally, we highlight ongoing research and future directions with the aim of improving arrhythmic risk assessment and management in AFD. This overview underscores the need for a multidisciplinary approach to optimize care and outcomes for patients with AFD.</p>","PeriodicalId":11225,"journal":{"name":"Diagnostics","volume":"15 2","pages":""},"PeriodicalIF":3.3000,"publicationDate":"2025-01-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11763368/pdf/","citationCount":"0","resultStr":"{\"title\":\"Anderson-Fabry Disease: An Overview of Current Diagnosis, Arrhythmic Risk Stratification, and Therapeutic Strategies.\",\"authors\":\"Chiara Tognola, Giacomo Ruzzenenti, Alessandro Maloberti, Marisa Varrenti, Patrizio Mazzone, Cristina Giannattasio, Fabrizio Guarracini\",\"doi\":\"10.3390/diagnostics15020139\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Anderson-Fabry disease (AFD) is a rare X-linked lysosomal storage disorder characterized by the accumulation of globotriaosylceramide, leading to multi-organ involvement and significant morbidity. Cardiovascular manifestations, particularly arrhythmias, are common and pose a considerable risk to affected individuals. This overview examines current approaches to arrhythmic risk stratification in AFD, focusing on the identification, assessment, and management of cardiac arrhythmias associated with the disease. We explore advancements in diagnostic techniques, including echocardiography, cardiac MRI, and ambulatory ECG monitoring, to enhance the detection of arrhythmogenic substrate. Furthermore, we discuss the role of genetic and biochemical markers in predicting arrhythmic risk and the implications for personalized treatment strategies. Current therapeutic interventions, including enzyme replacement therapy and antiarrhythmic medications, are reviewed in the context of their efficacy and limitations. Finally, we highlight ongoing research and future directions with the aim of improving arrhythmic risk assessment and management in AFD. This overview underscores the need for a multidisciplinary approach to optimize care and outcomes for patients with AFD.</p>\",\"PeriodicalId\":11225,\"journal\":{\"name\":\"Diagnostics\",\"volume\":\"15 2\",\"pages\":\"\"},\"PeriodicalIF\":3.3000,\"publicationDate\":\"2025-01-09\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11763368/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Diagnostics\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.3390/diagnostics15020139\",\"RegionNum\":3,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"MEDICINE, GENERAL & INTERNAL\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Diagnostics","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.3390/diagnostics15020139","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"MEDICINE, GENERAL & INTERNAL","Score":null,"Total":0}
引用次数: 0

摘要

安德森-法布里病(AFD)是一种罕见的x连锁溶酶体贮积性疾病,其特征是globotriaosylceramide的积累,导致多器官受累和显著的发病率。心血管表现,特别是心律失常,是常见的,并对受影响的个体构成相当大的风险。本文综述了AFD中心律失常风险分层的当前方法,重点关注与该疾病相关的心律失常的识别、评估和管理。我们探讨了诊断技术的进步,包括超声心动图、心脏MRI和动态心电图监测,以加强对心律失常底物的检测。此外,我们还讨论了遗传和生化标志物在预测心律失常风险中的作用以及对个性化治疗策略的影响。目前的治疗干预措施,包括酶替代疗法和抗心律失常药物,在其疗效和局限性的背景下进行了回顾。最后,我们强调了正在进行的研究和未来的方向,旨在改善AFD的心律失常风险评估和管理。本综述强调需要多学科方法来优化AFD患者的护理和结果。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
查看原文
分享 分享
微信好友 朋友圈 QQ好友 复制链接
本刊更多论文
Anderson-Fabry Disease: An Overview of Current Diagnosis, Arrhythmic Risk Stratification, and Therapeutic Strategies.

Anderson-Fabry disease (AFD) is a rare X-linked lysosomal storage disorder characterized by the accumulation of globotriaosylceramide, leading to multi-organ involvement and significant morbidity. Cardiovascular manifestations, particularly arrhythmias, are common and pose a considerable risk to affected individuals. This overview examines current approaches to arrhythmic risk stratification in AFD, focusing on the identification, assessment, and management of cardiac arrhythmias associated with the disease. We explore advancements in diagnostic techniques, including echocardiography, cardiac MRI, and ambulatory ECG monitoring, to enhance the detection of arrhythmogenic substrate. Furthermore, we discuss the role of genetic and biochemical markers in predicting arrhythmic risk and the implications for personalized treatment strategies. Current therapeutic interventions, including enzyme replacement therapy and antiarrhythmic medications, are reviewed in the context of their efficacy and limitations. Finally, we highlight ongoing research and future directions with the aim of improving arrhythmic risk assessment and management in AFD. This overview underscores the need for a multidisciplinary approach to optimize care and outcomes for patients with AFD.

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
Diagnostics
Diagnostics Biochemistry, Genetics and Molecular Biology-Clinical Biochemistry
CiteScore
4.70
自引率
8.30%
发文量
2699
审稿时长
19.64 days
期刊介绍: Diagnostics (ISSN 2075-4418) is an international scholarly open access journal on medical diagnostics. It publishes original research articles, reviews, communications and short notes on the research and development of medical diagnostics. There is no restriction on the length of the papers. Our aim is to encourage scientists to publish their experimental and theoretical research in as much detail as possible. Full experimental and/or methodological details must be provided for research articles.
期刊最新文献
Forecasting Patient-Specific Abdominal Aortic Aneurysm Geometry with Mixed-Effects Models. Gestational Age-Specific Biometric and Estimated Fetal Weight Curves in Gastroschisis: A Brazilian Multicenter Cohort Study. Bacterial Gastrointestinal Infections in Pediatric Inflammatory Bowel Disease (PIBD)-A Single-Center Experience of Epidemiology, Management, and Outcome. Contrast Enhancement Is Associated with a Higher DSC MRI-Derived Cerebral Metabolic Rate of Oxygen Index in Untreated Glioblastoma. Estimating the Post-Mortem Interval Under Extreme Heat Environments: A Climate-Adaptive Case Series Based on Artificial Intelligence-Supported Diagnostics.
×
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
现在去查看 取消
×
提示
确定
0
微信
客服QQ
Book学术公众号 扫码关注我们
反馈
×
意见反馈
请填写您的意见或建议
请填写您的手机或邮箱
已复制链接
已复制链接
快去分享给好友吧!
我知道了
×
扫码分享
扫码分享
Book学术官方微信
Book学术官方微信
Book学术文献互助
Book学术文献互助群
群 号:604180095
Book学术
文献互助 智能选刊 最新文献 互助须知 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 Book学术 All rights reserved.
ghs 京公网安备 11010802042870号 京ICP备2023020795号-1