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家族性地中海热(MEFV)基因突变在经活检证实的原发性肾小球肾炎患者中的频率。

Frequency of familial Mediterranean fever (MEFV) gene mutations in patients with biopsy-proven primary glomerulonephritis.

机构信息

Division of Nephrology, Department of Internal Medicine, Faculty of Medicine, Cumhuriyet University, Sivas, Turkey.

Department of Medical Genetics, Faculty of Medicine, Cumhuriyet University, Sivas, Turkey.

出版信息

Clin Rheumatol. 2017 Nov;36(11):2589-2594. doi: 10.1007/s10067-017-3701-y. Epub 2017 Jun 1.

Abstract

Primary glomerulopathies are those disorders that affect glomerular structure, function, or both in the absence of a multisystem disorder. We aimed to evaluate the frequency of MEFV gene mutation to show possible coexistence of FMF in patients diagnosed with biopsy-proven primary glomerulonephritis (GN). A total of 64 patients with biopsy-proven primary GN were included in the study. MEFV gene mutations examined retrospectively. The mean age of patients was 39.6 ± 13.4 (range 18-69), 35 of patients were female and 29 of patients were male. Of the 64 patients, 17 were mesangial proliferative glomerulonephritis (MsPGN), 15 were IgA nephropathy (IgAN), 12 were membranous glomerulonephritis (MGN), 11 were focal segmental glomerulosclerosis (FSGS), three were membranous proliferative glomerulonephritis (MPGN), three were immune complex glomerulonephritis (ICGN), two were minimal change disease (MCD), and one was IgM nephropathy (IgMN). MEFV gene mutation was detected in 35.9% (23) of these patients. The most frequently detected mutations were E148Q and M694V. Twelve cases (18.75% of GN patients) with MEFV gene mutation were diagnosed as FMF phenotype I. The frequency of MEFV gene mutation was detected at a high rate of 35.9%. Further studies with larger populations are needed to clarify the importance of these mutations on clinical progression of glomerulonephritis.

摘要

原发性肾小球疾病是指在无多系统疾病的情况下影响肾小球结构、功能或两者的疾病。我们旨在评估 MEFV 基因突变的频率,以显示在经活检证实的原发性肾小球肾炎 (GN) 患者中可能存在 FMF 的共存。总共纳入了 64 名经活检证实的原发性 GN 患者。回顾性检查 MEFV 基因突变。患者的平均年龄为 39.6±13.4(范围 18-69),35 名患者为女性,29 名患者为男性。在 64 名患者中,17 名患有系膜增生性肾小球肾炎 (MsPGN)、15 名患有 IgA 肾病 (IgAN)、12 名患有膜性肾小球肾炎 (MGN)、11 名患有局灶节段性肾小球硬化症 (FSGS)、3 名患有膜增生性肾小球肾炎 (MPGN)、3 名患有免疫复合物性肾小球肾炎 (ICGN)、2 名患有微小病变性肾病 (MCD) 和 1 名患有 IgM 肾病 (IgMN)。在这些患者中,检测到 MEFV 基因突变的比例为 35.9%(23 例)。最常见的突变是 E148Q 和 M694V。12 例(GN 患者的 18.75%)具有 MEFV 基因突变的患者被诊断为 FMF 表型 I。MEFV 基因突变的频率检测率很高,为 35.9%。需要进一步进行更大规模的研究,以阐明这些突变对肾小球肾炎临床进展的重要性。

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