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单克隆免疫球蛋白沉积性增生性肾小球肾炎病例系列

A Case Series of Monoclonal Immunoglobulin-Depositing Proliferative Glomerulonephritis.

作者信息

Venkatesh Leelavathi, Shetty Bhushan Chandrahasa

机构信息

Department of Nephrology, Institute of Nephrourology (INU) Bangalore, Karnataka, India.

Department of Nephrology, Kasturba Medical College Mangalore, Manipal Academy of Higher Education, Manipal, Karnataka, India.

出版信息

Indian J Nephrol. 2024 Sep-Oct;34(5):507-509. doi: 10.25259/ijn_572_23. Epub 2024 Jul 8.

Abstract

Proliferative glomerulonephritis with monoclonal immunoglobulin deposition disease (PGNMIDD) is a rare entity. We evaluated the clinicopathological features of PGNMIDD and the effectiveness of different treatment regimens in 13 cases diagnosed using kidney biopsy. Most had chronic kidney disease followed by acute nephritic syndrome, rapidly progressive glomerulonephritis, and nephrotic syndrome. Membranoproliferative glomerulonephritis was the most common pattern of renal injury. Three patients had abnormal bone marrow studies. Different treatment regimens were deployed; >60% had partial remission at the end of six months and 30.7% progressed to end stage renal disease.

摘要

增殖性肾小球肾炎伴单克隆免疫球蛋白沉积病(PGNMIDD)是一种罕见的疾病。我们评估了13例经肾活检确诊的PGNMIDD患者的临床病理特征及不同治疗方案的疗效。大多数患者表现为慢性肾脏病,其次为急性肾炎综合征、快速进展性肾小球肾炎和肾病综合征。膜增生性肾小球肾炎是最常见的肾损伤模式。3例患者骨髓检查异常。采用了不同的治疗方案;超过60%的患者在6个月末达到部分缓解,30.7%的患者进展至终末期肾病。

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Proliferative glomerulonephritis with monoclonal IgG deposits.伴有单克隆IgG沉积的增殖性肾小球肾炎
J Am Soc Nephrol. 2009 Sep;20(9):2055-64. doi: 10.1681/ASN.2009010110. Epub 2009 May 21.

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