单克隆肾病(MGRS)--喀拉拉邦一家三级医疗中心的病例系列。

IF 0.8 Q4 UROLOGY & NEPHROLOGY
Indian Journal of Nephrology Pub Date : 2024-01-01 Epub Date: 2023-03-20 DOI:10.4103/ijn.ijn_329_22
Priya Padmanabhan, Jayameena P, Neena Mampilly, Shinto Francis, Ajmal Sherif, Sunil George, Thomas Mathew
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引用次数: 0

摘要

肾脏重症单克隆抗体病(MGRS)的重要性日益凸显,因为识别单克隆沉积并加以治疗,而不是将肾功能障碍作为主要病理治疗,从而避免了患者发展为终末期肾病。由于该病影响的是老年人群,因此有可能误诊为心肾综合征。我们介绍了本中心确诊的四例 MGRS 患者。他们出现蛋白尿或不明原因的肾功能障碍。其中三名患者被确诊为淀粉样变性,其中两人患有λ型淀粉样变性,一人患有卡帕型淀粉样变性。第四名患者患有纤维性肾小球肾炎,伴有 kappa 限制,进一步评估后诊断为慢性淋巴细胞白血病。如果蛋白质电泳中没有 "M "带,骨髓检查也正常,医生不应停止进一步评估。定量血清免疫固定电泳和肾活检的电子显微镜检查已成为此类患者的综合诊断工具。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Monoclonal Gammopathy of Renal Significance (MGRS) - Case Series from a Tertiary Center in Kerala.

Monoclonal gammopathy of renal significance (MGRS) has gained importance because identifying the monoclonal deposit and addressing it, rather than treating renal dysfunction as the primary pathology, has salvaged the patients from progressing into end-stage renal disease. Since it affects elderly population, there could be a propensity to misdiagnose them with cardiorenal syndrome. We present four patients of MGRS diagnosed from our center. They presented with proteinuria or unexplained renal dysfunction. Three of the patients were diagnosed to have amyloidosis, of which two had lambda-type and one had kappa amyloidosis. The fourth patient had fibrillary glomerulonephritis with kappa restriction, further evaluation of which led to diagnosis of chronic lymphocytic leukemia. Absence of "M" band in protein electrophoresis and a normal bone marrow study should not stop physicians from further evaluation. Quantitative serum immunofixation electrophoresis and electron microscopic examination of renal biopsy have become a comprehensive diagnostic tool in such patients.

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来源期刊
Indian Journal of Nephrology
Indian Journal of Nephrology UROLOGY & NEPHROLOGY-
CiteScore
1.40
自引率
0.00%
发文量
128
审稿时长
24 weeks
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