法布里病:一种需要个性化医疗的罕见疾病。

IF 1.8 4区 医学 Q3 UROLOGY & NEPHROLOGY International Urology and Nephrology Pub Date : 2024-10-01 Epub Date: 2024-04-13 DOI:10.1007/s11255-024-04042-4
Sarah Lerario, Luigi Monti, Irene Ambrosetti, Agnese Luglio, Andrea Pietra, Valeria Aiello, Francesca Montanari, Antonio Bellasi, Gianluigi Zaza, Antonio Galante, Davide Salera, Irene Capelli, Gaetano La Manna, Michele Provenzano
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引用次数: 0

摘要

法布里病(Fabry Disease,FD)是一种因溶酶体半乳糖苷酶 A(α-GalA)活性缺乏而引起的遗传病,这种酶负责球糖基甘油三酯(Gb3)的分解代谢。由于溶酶体遍布全身,并在分解和回收胞浆化合物方面发挥着重要作用,FD 可影响多个器官并导致各种症状,包括肾脏、心血管、神经、皮肤和眼部表现。由于 FD 的症状无特异性且罕见,通常在晚期才被诊断出来。然而,酶替代疗法(ERT)和伴侣疗法等靶向疗法的引入,通过恢复α-GalA酶的活性,大大改善了FD的自然病史和预后。尽管取得了这些进展,但目前可用的疗法仍存在局限性,这促使人们研究新的潜在治疗方法,包括替代酶替代疗法、底物减少疗法、mRNA疗法和基因疗法。在这篇综述中,我们分析了 FD 的流行病学、病理生理学和治疗方法,并特别强调了一些有希望的治疗机会,这些机会可能会使这种罕见疾病的治疗很快从标准化方法转变为个性化方法。
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Fabry disease: a rare disorder calling for personalized medicine.

Fabry Disease (FD) is a genetic disease caused by a deficiency in the activity of lysosomal galactosidase A (α-GalA), an enzyme responsible for the catabolism of globotriaosylceramide (Gb3). Since lysosomes are present throughout the body and play a crucial role in catabolism and recycling of cytosolic compounds, FD can affect multiple organs and result in various symptoms, including renal, cardiovascular, neurological, cutaneous, and ophthalmic manifestations. Due to the nonspecific symptoms and the rarity of FD, it is often diagnosed late in life. However, introducing targeted therapies such as enzyme replacement therapy (ERT) and chaperone therapy has significantly improved FD's natural history and prognosis by restoring α-GalA enzyme activity. Despite the advancements, there are limitations to the currently available therapies, which has prompted research into new potential treatments for FD, including alternative forms of enzyme replacement therapy, substrate reduction therapy, mRNA therapy, and genetic therapy. In this review, we analyze the epidemiology, pathophysiology, and treatment of FD, with particular emphasis on promising therapeutic opportunities that could shift the treatment of this rare disease from a standardized to a personalized approach soon.

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来源期刊
International Urology and Nephrology
International Urology and Nephrology 医学-泌尿学与肾脏学
CiteScore
3.40
自引率
5.00%
发文量
329
审稿时长
1.7 months
期刊介绍: International Urology and Nephrology publishes original papers on a broad range of topics in urology, nephrology and andrology. The journal integrates papers originating from clinical practice.
期刊最新文献
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