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[膀胱孤立性原发性淀粉样变性]

[Isolated primary amyloidosis of the bladder].

作者信息

Chamatan Ahmad, Peters Catherine, Ngendahayo Placide, Maquet Jean-Hubert

机构信息

Service d'Urologie, Centre Hospitalier Hornu-Frameries, Mons, Belgique.

出版信息

Prog Urol. 2004 Apr;14(2):218-20.

Abstract

Amyloidosis is a group of storage diseases concerning various proteins, classified as a function of the type of protein. Amyloidosis of the bladder is a rare disease (about one hundred cases have been reported over the last thirty years), but the bladder appears to be the most frequent site in the urinary tract. Amyloidosis may be either primary or secondary, isolated or involving multiple organs. Complementary investigations must be performed to eliminate systemic involvement before proposing the diagnosis of isolated amyloid and analysis of the type of amyloid can help target these investigations. In most cases, the patient presents with painless macroscopic haematuria. The diagnosis is always based on histological examination of cystoscopy samples. In the light of a new case of isolated amyloidosis of the bladder diagnosed in their department in a context of assessment of microscopic haematuria and after a review of the literature, the authors discuss the diagnostic features, the examinations designed to exclude systemic disease and the various treatments.

摘要

淀粉样变性是一组涉及多种蛋白质的贮积病,根据蛋白质类型进行分类。膀胱淀粉样变性是一种罕见疾病(过去三十年报告了约100例),但膀胱似乎是泌尿道中最常受累的部位。淀粉样变性可为原发性或继发性,可为孤立性或累及多个器官。在诊断孤立性淀粉样变性之前,必须进行补充检查以排除系统性受累,淀粉样蛋白类型分析有助于确定这些检查。大多数情况下,患者表现为无痛性肉眼血尿。诊断始终基于膀胱镜检查样本的组织学检查。鉴于在他们科室诊断出的一例孤立性膀胱淀粉样变性新病例,该病例是在对镜下血尿进行评估的背景下诊断出来的,并且在查阅文献之后,作者讨论了诊断特征、旨在排除全身性疾病的检查以及各种治疗方法。

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