Renal involvement in familial Mediterranean fever in an Algerian population.

IF 1.1 4区 医学 Q3 UROLOGY & NEPHROLOGY Clinical nephrology Pub Date : 2023-04-01 DOI:10.5414/CN110930
Ghalia Khellaf, Ali Benziane, Louiza Kaci, Djouher Ait-Idir, Soumia Missoum, Mohamed Benabadji
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引用次数: 1

Abstract

The objectives of this study were to investigate the clinical biological and histological renal involvement secondary to familial Mediterranean fever (FMF), the epidemiological data, genetics of our patients and their evolution under treatment. We prospectively studied 58 Algerian patients admitted in our nephrology department from January 2012 to January 2021. The diagnosis of nephropathy was suspected clinically and biologically and confirmed histologically. All our patients were tested for MEFV mutations. Results: 58 patients, 30 males and 28 females, mean age 31.68 ± 12.71; 3 (5.17%) chronic dialysis patients and 55 (94.82%) referred to the nephrology department for renal biopsy with renal symptomatology consisting of nephrotic syndrome in 50 (94. 73%), associated with renal failure 27 (47.36%), mainly primary in 23 (34.5%), secondary to seronegative lupus 13 (22.4%), Crohn's disease 9 (14.5%), sarcoidosis 3 (5.26%), and lymphoma 1 (1.7%); 29 (50%) were from consangineous marriages, the histological study found AA amyloidosis in 52 (89.6%); the genetic study confirmed the diagnosis of FMF in 58 (100%). The evolution of the patients: 20 (34.48%) followed in consultation, 25 (43.10%) in hemodialysis and 13 (22.41%) deceased. Conclusion: Renal involvement was the revealing complication in the diagnosis of FMF which exists in our country, and is still underdiagnosed.

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阿尔及利亚人群家族性地中海热中肾脏受累。
本研究的目的是调查家族性地中海热(FMF)继发于肾脏的临床生物学和组织学损害、流行病学资料、患者的遗传学及其在治疗期间的演变。我们前瞻性研究了2012年1月至2021年1月在我们肾脏病科住院的58名阿尔及利亚患者。肾病的诊断在临床和生物学上被怀疑,并在组织学上被证实。我们所有的病人都进行了MEFV突变检测。结果:58例患者,男30例,女28例,平均年龄31.68±12.71岁;慢性透析患者3例(5.17%),肾活检患者55例(94.82%),肾脏症状包括肾病综合征50例(94例)。73%),伴肾功能衰竭27例(47.36%),主要原发23例(34.5%),继发血清阴性狼疮13例(22.4%),克罗恩病9例(14.5%),结节病3例(5.26%),淋巴瘤1例(1.7%);同源婚姻29例(50%),组织学检查发现AA淀粉样变性52例(89.6%);遗传学研究证实58例(100%)诊断为FMF。随访20例(34.48%),血液透析25例(43.10%),死亡13例(22.41%)。结论:肾脏受累是我国FMF诊断的重要并发症,但仍未得到充分诊断。
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来源期刊
Clinical nephrology
Clinical nephrology 医学-泌尿学与肾脏学
CiteScore
2.10
自引率
9.10%
发文量
138
审稿时长
4-8 weeks
期刊介绍: Clinical Nephrology appears monthly and publishes manuscripts containing original material with emphasis on the following topics: prophylaxis, pathophysiology, immunology, diagnosis, therapy, experimental approaches and dialysis and transplantation.
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