Suppr超能文献

[一例安德森-法布里病:诊断与随访的多学科方法]

[A case of Anderson-Fabry disease: a multidisciplinary approach for diagnosis and follow up].

作者信息

Zito Anna, De Pascalis Antonio, Armeni Annarita, Ria Paolo, Barbarini Leonardo, Caggiula Marcella, My Filomena, Barbarini Silvia, Trianni Giorgio, Napoli Marcello

机构信息

Unità Operativa Nefrologia e Dialisi, Ospedale Vito Fazzi, Lecce.

Unità Operativa Neurologia, Ospedale Vito Fazzi, Lecce.

出版信息

G Ital Nefrol. 2018 Sep;35(5).

Abstract

Fabry disease (also known as Anderson-Fabry disease, angiocheratoma corporis diffusum, diffuse angiocheratoma) is a rare tesaurismosis linked to the deficiency of the lysosomal enzyme alpha-galactosidase A, required for the physiological catabolism of glycosphingolipids. The related clinical signs show a multisystemic feature and define a degenerative and disabling pathology, whose approach requires a close multidisciplinary specialist collaboration. Currently, the renewed interest in the disease is aimed at the need to provide an early diagnosis, in order to early begin the enzyme replacement therapy and to slow down or avoid the establishment of irreparable organ damage. For this reason, the diagnostic suspicion becomes crucial and arises from the careful observation and research of the symptoms, together with the anamnesis and the overall clinical evaluation of the patient.

摘要

法布里病(也称为安德森 - 法布里病、弥漫性躯体血管角质瘤、弥漫性血管角质瘤)是一种罕见的贮积症,与溶酶体酶α - 半乳糖苷酶A缺乏有关,该酶是糖鞘脂生理分解代谢所必需的。相关临床症状具有多系统特征,定义了一种退行性和致残性病理状况,其治疗需要多学科专家密切协作。目前,对该疾病重新产生的兴趣旨在满足早期诊断的需求,以便尽早开始酶替代疗法,并减缓或避免不可修复的器官损伤的发生。因此,诊断怀疑至关重要,它源于对症状的仔细观察和研究,以及患者的病史和全面临床评估。

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验