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肾淀粉样变性:来自一个肾脏病中心的成人流行病学、临床及实验室特征

Renal Amyloidosis: Epidemiological, Clinical, and Laboratory Profile in Adults from One Nephrology Center.

作者信息

Kaaroud Hayet, Harzallah Amel, Hajji Mariem, Chargui Soumaya, Barbouch Samia, Turki Sami, Trabelsi Raja, Goucha Rim, Ben Moussa Fatma, Ben Maiz Hedi, Ben Hamida Fethi, Abderrahim Ezzeddine

机构信息

Department of Medicine A, Charles Nicolle Hospital, Tunis, Tunisia.

Laboratory of Renal Pathology LR00SP01, Charles Nicolle Hospital, Tunis, Tunisia.

出版信息

Int J Nephrol. 2022 Jul 18;2022:8493479. doi: 10.1155/2022/8493479. eCollection 2022.

Abstract

BACKGROUND

Renal amyloidosis is one of the main differential diagnoses of nephrotic proteinuria in adults and the elderly. The aim of this study with the most important series in our country is to contribute to the epidemiological, clinical, and etiological study of the renal amyloidosis.

METHODS

In a retrospective study carried out between 1975 and 2019, 310 cases of histologically proven and typed renal amyloidosis were selected for this study.

RESULTS

There were 209 men and 101 women with a mean age of 53.8 ± 15.4 years (range, 17-84 years). Of the 310 cases, 255 (82.3%) were diagnosed with AA renal amyloidosis and 55 (17.7%) with non-AA amyloidosis. Infections were the main cause of AA amyloidosis, and tuberculosis was the most frequent etiology. The period from the onset of the underlying disease to diagnosis of the renal amyloidosis was an average of 177 months. The most frequent manifestations at the time of diagnosis were nephrotic syndrome (84%), chronic renal failure (30.3%), and end-stage renal disease (37.8%). After a medium follow-up of 16 months (range, 0-68 months), mortality occurred in 60 cases.

CONCLUSIONS

Given the high frequency of AA amyloidosis in our country, awareness of the proper management of infectious and chronic inflammatory diseases remains a priority in reducing the occurrence of this serious disease.

摘要

背景

肾淀粉样变性是成人和老年人肾病性蛋白尿的主要鉴别诊断之一。本研究以我国最重要的系列病例为对象,旨在为肾淀粉样变性的流行病学、临床和病因学研究做出贡献。

方法

在一项于1975年至2019年期间开展的回顾性研究中,选取了310例经组织学证实且分型的肾淀粉样变性病例进行本研究。

结果

共有209例男性和101例女性,平均年龄为53.8±15.4岁(范围为17 - 84岁)。在这310例病例中,255例(82.3%)被诊断为AA型肾淀粉样变性,55例(17.7%)为非AA型淀粉样变性。感染是AA型淀粉样变性的主要原因,结核病是最常见的病因。从基础疾病发病到肾淀粉样变性诊断的时间平均为177个月。诊断时最常见的表现为肾病综合征(84%)、慢性肾衰竭(30.3%)和终末期肾病(37.8%)。经过平均16个月(范围为0 - 68个月)的随访,60例患者死亡。

结论

鉴于我国AA型淀粉样变性的高发病率,认识到对感染性和慢性炎症性疾病的恰当管理仍是降低这种严重疾病发生率的首要任务。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a09e/9314002/2f149b2d0595/IJN2022-8493479.001.jpg

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