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印度的C1q肾病:一项单中心研究。

C1q nephropathy in India: a single-center study.

作者信息

Kanodia K V, Vanikar A V, Patel R D, Suthar K S, Patel H V, Gumber M A, Shah P R, Trivedi H L

机构信息

Department of Pathology, Laboratory Medicine and Transfusion Services and Immunohematology, Institute of Kidney Diseases and Research Centre and Institute of Transplantation Sciences, Civil Hospital Campus, Asarwa, Ahmedabad, India.

出版信息

Saudi J Kidney Dis Transpl. 2015 Mar;26(2):398-403. doi: 10.4103/1319-2442.152562.

Abstract

C1q nephropathy (C1qN) is defined by conspicuous C1q deposits in the glomerular mesangial regions of patients who do not have any evidence of systemic lupus erythematosus (SLE). We present our experience with C1qN over the last three years. In total, 1775 native renal biopsies were reviewed and dominant/co-dominant C1q mesangial deposits in patients with absence of clinical and/or serological evidence of SLE were considered as C1qN. Their clinical profile and renal function status were studied and correlated. C1qN was observed in 11 patients (0.61%), and included eight males and three females; the mean age was 36.6 years. The most common presentation was nephrotic syndrome. Hematuria was noted in eight patients (72%). The mean serum creatinine was 2.78 mg/dL. Hypertension was seen in two patients (18%). Mesangial proliferative glomerulonephritis (MePGN) was the most common histological pattern, followed by focal and segmental glomerulosclerosis and other lesions. The common codeposits along with C1q were IgM, followed by C3 and others. MePGN had better prognosis than others. To conclude, C1qN was noted in 0.61% of all renal biopsies with bimodal age distribution and may present as podocytopathy or non-podocytopathy. The prognosis depends on the morphological pattern and C1q deposits per se are not prognostic indicators.

摘要

C1q肾病(C1qN)的定义是,在没有任何系统性红斑狼疮(SLE)证据的患者肾小球系膜区域有明显的C1q沉积。我们介绍过去三年来我们对C1qN的经验。总共回顾了1775例肾活检病例,将没有SLE临床和/或血清学证据的患者中占主导/共主导的C1q系膜沉积视为C1qN。对他们的临床特征和肾功能状况进行了研究并进行关联分析。11例患者(0.61%)被诊断为C1qN,其中男性8例,女性3例;平均年龄为36.6岁。最常见的表现是肾病综合征。8例患者(72%)有血尿。平均血清肌酐为2.78mg/dL。2例患者(18%)有高血压。系膜增生性肾小球肾炎(MePGN)是最常见的组织学类型,其次是局灶节段性肾小球硬化和其他病变。与C1q共同沉积的常见物质是IgM,其次是C3和其他物质。MePGN的预后比其他类型好。总之,在所有肾活检病例中,0.61%被诊断为C1qN,其年龄分布呈双峰,可表现为足细胞病或非足细胞病。预后取决于形态学类型,C1q沉积本身不是预后指标。

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