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[一名中年男性同时出现持续性血尿(薄基底膜肾病)和轻链蛋白尿(良性单克隆丙种球蛋白病)]

[Simultaneous occurrence of persistent hematuria (thin basement membrane nephropathy) and light-chain proteinuria (benign monoclonal gammopathy) in a middle-aged male].

作者信息

Iványi B, Mohácsi G, Sonkodi S, Ormos J

机构信息

Szent-Györgyi Albert Orvostudományi Egyetem, Szeged.

出版信息

Orv Hetil. 1992 Sep 20;133(38):2431-4.

PMID:1408078
Abstract

IgG lambda type of monoclonal gammopathy and thin basement membrane nephropathy were established in a middle-aged man examined because of persistent haematuria, lambda light-chain proteinuria and moderately diminished renal function. A 10% level of plasmocytosis was verified by bone-marrow aspiration. The more than 6-year follow-up showed the gammopathy to be benign. The thin basement membrane nephropathy was verified by electronmicroscopic analysis of renal tissue obtained by percutaneous renal biopsy: lamina densa of the glomerular capillaries thinned to 30-100 nm. In spite of the usually good outcome of thin basement membrane nephropathy, in this case it was accompanied by glomerular sclerosis, subsequent destruction of nephrons, hypertensive vascular alterations and a clinical deterioration of the renal function after 4 years. A rebiopsy excluded the possible complications (amyloidosis, non-amyloid immunoglobulin nephropathy, cylinder nephropathy, etc) of light-chain proteinuria.

摘要

一名中年男性因持续性血尿、λ轻链蛋白尿和肾功能中度减退接受检查,确诊为IgG λ型单克隆丙种球蛋白病和薄基底膜肾病。经骨髓穿刺证实浆细胞增多症水平为10%。超过6年的随访显示丙种球蛋白病为良性。通过经皮肾活检获取的肾组织进行电子显微镜分析,证实为薄基底膜肾病:肾小球毛细血管的致密层变薄至30 - 100纳米。尽管薄基底膜肾病通常预后良好,但在该病例中,4年后出现了肾小球硬化、随后的肾单位破坏、高血压性血管改变以及肾功能的临床恶化。再次活检排除了轻链蛋白尿可能的并发症(淀粉样变性、非淀粉样免疫球蛋白肾病、管型肾病等)。

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