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单克隆丙种球蛋白病与伴有有组织微管沉积物的肾小球肾炎。

Monoclonal gammopathy and glomerulonephritis with organized microtubular deposits.

作者信息

Rollino C, Coppo R, Mazzucco G, Roccatello D, Amore A, Basolo B, Martina G, Gianoglio B, Piccoli G

机构信息

Institute of Nephro-Urology, University of Turin, Italy.

出版信息

Am J Kidney Dis. 1990 Mar;15(3):276-80. doi: 10.1016/s0272-6386(12)80775-x.

DOI:10.1016/s0272-6386(12)80775-x
PMID:2106261
Abstract

We report a case with IgG-kappa monoclonal gammopathy of unidentified significance (MGUS) and glomerulonephritis (GN) with organized microtubular deposits on electron microscopy (EM). Light microscopy (LM) examination showed exudative features and moderate extracapillary proliferation. An acute nephritic syndrome with a rapidly progressive renal failure was clinically manifest at the onset and during each relapse. The patient was treated with methylprednisolone pulses followed by oral prednisone, cyclophosphamide, plasmapheresis, and maintenance courses of chemotherapy. The response to treatment was good, with a temporary improvement of renal function and control of the downhill course over a 3-year follow-up.

摘要

我们报告一例具有意义未明的IgG-κ单克隆丙种球蛋白病(MGUS)和肾小球肾炎(GN)的病例,电镜检查发现有组织化的微管沉积物。光镜(LM)检查显示渗出性特征和中度的毛细血管外增生。临床上起病时及每次复发时均表现为急性肾炎综合征伴快速进展的肾衰竭。患者接受了甲泼尼龙冲击治疗,随后口服泼尼松、环磷酰胺、血浆置换及化疗维持疗程。治疗反应良好,在3年的随访中肾功能暂时改善,病情的恶化进程得到控制。

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Monoclonal gammopathy and glomerulonephritis with organized microtubular deposits.单克隆丙种球蛋白病与伴有有组织微管沉积物的肾小球肾炎。
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引用本文的文献

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Development of Renal Failure without Proteinuria in a Patient with Monoclonal Gammopathy of Undetermined Significance: An Unusual Presentation of AL Kappa Amyloidosis.意义未明的单克隆丙种球蛋白病患者出现无蛋白尿的肾衰竭:AL κ轻链淀粉样变性的一种不寻常表现
Case Rep Nephrol. 2012;2012:573650. doi: 10.1155/2012/573650. Epub 2012 Oct 2.
2
Molecular study of an IgG1kappa cryoglobulin yielding organized microtubular deposits and glomerulonephritis in the course of chronic lymphocytic leukaemia.慢性淋巴细胞白血病过程中产生有组织微管沉积物和肾小球肾炎的IgG1κ冷球蛋白的分子研究
Clin Exp Immunol. 2002 Jul;129(1):113-8. doi: 10.1046/j.1365-2249.2002.01896.x.