膀胱直链淀粉病:罕见的位置。关于一个案例

IF 0.7 4区 医学 Q4 UROLOGY & NEPHROLOGY Nephrologie & Therapeutique Pub Date : 2022-12-01 DOI:10.1016/j.nephro.2022.10.004
Nisrine Hikki , Samia Sassi , Naji Reguieg , Kaoutar Znati , Tarik Bouattar , Loubna Benamar , Rabia Bayahia , Naima Ouzeddoun
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引用次数: 0

摘要

摘要AA淀粉样变的膀胱定位是罕见的。它可以导致大量和反复的血尿。我们报告一例继发于克罗恩病的膀胱AA淀粉样变。临床观察男性,62岁,1991年发病,克罗恩病合并肾AA淀粉样变。20年前,他接受了一名HLA相同的捐赠者的肾脏移植手术。经过18年的临床缓解期后,患者因间歇性腹泻形式的克罗恩病突然发作而入院。用皮质类固醇治疗使病情有了良好的发展。一年后,他因肉眼可见的大量血尿再次住院。膀胱活检组织学检查显示AA淀粉样变。患者完全康复,但6周后死于泌尿源性感染性休克。结论AA型淀粉样变性膀胱定位的治疗应以病因为基础。血尿有时是大量的,特殊情况下需要紧急膀胱切除术止血。
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Amylose AA vésicale : une localisation rare. À propos d’un cas

Introduction

Bladder localization of AA amyloidosis is rare. It can be responsible for massive and recurrent hematuria. We report a case of bladder AA amyloidosis secondary to Crohn's disease in a renal transplant patient.

Clinical observation

A 62-year-old man, suffering from Crohn's disease since 1991 complicated by renal AA amyloidosis. He received a kidney transplant since 20 years from an HLA identical donor. After an 18-year period of clinical remission, the patient was admitted for a flare-up of his Crohn's disease in the form of intermittent diarrhoea. Treatment with corticosteroids allowed a good evolution. A year later, he was rehospitalized for massive macroscopic haematuria. Histological examination of the bladder biopsy revealed AA amyloidosis. The patient fully recovered but died 6 weeks later from septic shock of urinary origin.

Conclusion

The treatment of bladder localization of AA amyloidosis is based on treating the cause. Hematuria is sometimes massive, exceptionally requiring emergency cystectomy for haemostasis.

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来源期刊
Nephrologie & Therapeutique
Nephrologie & Therapeutique 医学-泌尿学与肾脏学
CiteScore
0.80
自引率
14.30%
发文量
485
审稿时长
11.9 weeks
期刊介绍: Organe d''expression de la Société de Néphrologie, de la Société Francophone de Dialyse et de la Société de Néphrologie Pédiatrique, Néphrologie et Thérapeutique a pour vocation de publier des textes en français dans le domaine de la Néphrologie, qu''il s''agisse d''actualisation des connaissances, de recommandations de bonne pratique clinique, de publications originales, ou d''informations sur la vie des trois sociétés fondatrices. La variété des thèmes abordés reflète la richesse de la Néphrologie, qu''il s''agisse d''aspects fondamentaux issus de la physiologie, de l''immunologie, de l''anatomo-pathologie, ou de la génétique, ou de sujets de néphrologie clinique, notamment ceux en rapport avec les thérapeutiques néphrologiques, transplantation, hémodialyse et dialyse péritonéale.
期刊最新文献
Posters Posters commentés Communications orales [Biological parameters for assessment and monitoring anemia in hemodialysis patients]. [Arguments for the use of cardiac biomarkers in hemodialysis: natriuretic peptides and troponins].
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