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轻链相关肾病中近端肾小管的形态学改变。

Morphologic alterations of the proximal tubules in light chain-related renal disease.

作者信息

Sanders P W, Herrera G A, Lott R L, Galla J H

机构信息

Nephrology Research and Training Center, University of Alabama, Birmingham.

出版信息

Kidney Int. 1988 Apr;33(4):881-9. doi: 10.1038/ki.1988.80.

Abstract

Previous studies from our laboratory have shown that human immunoglobulin light chains (LC) can be toxic to the epithelium of the rat proximal tubule. To examine the toxicity of monoclonal LC's in man, 11 kidney specimens (EXP group) obtained from patients with monotypical LC-related renal disease (7 lambda, 4 kappa), documented by the presence of monoclonal LC's in the serum or urine and in the tissue, were examined by light, immunofluorescence, electron, and immunoelectron microscopy. This EXP group had monotypical LC deposition in the tubules and/or the glomeruli and did not have evidence of intraluminal LC precipitation and cast formation, which alters tubule morphology. A control group (CON; N = 12) of kidney specimens was obtained from patients who had proteinuria greater than 2.5 g/24 hr and mean age (49 +/- 4 vs. 59 +/- 3 years; P = NS), serum creatinine concentration (2.4 +/- 0.5 vs. 3.2 +/- 1.5 mg/dl; P = NS) and creatinine clearance (65 +/- 13 vs. 63 +/- 12 ml/min; P = NS) similar to the EXP group. All of the EXP specimens demonstrated varying degrees of proximal tubule damage, manifested by cell vacuolation, desquamation, loss of the luminal brush border, and, often, coagulation necrosis.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

我们实验室之前的研究表明,人免疫球蛋白轻链(LC)可对大鼠近端小管上皮产生毒性。为了检测单克隆LC对人类的毒性,对11份肾脏标本(实验组)进行了检查,这些标本取自单型LC相关肾病患者(7例λ型,4例κ型),血清、尿液及组织中均存在单克隆LC,通过光镜、免疫荧光、电子显微镜及免疫电子显微镜进行观察。该实验组在肾小管和/或肾小球中有单型LC沉积,且无管腔内LC沉淀和管型形成的证据,而管腔内LC沉淀和管型形成会改变肾小管形态。对照组(CON;n = 12)的肾脏标本取自蛋白尿大于2.5 g/24小时的患者,其平均年龄(49±4岁对59±3岁;P =无显著性差异)、血清肌酐浓度(2.4±0.5对3.2±1.5 mg/dl;P =无显著性差异)及肌酐清除率(65±13对63±12 ml/分钟;P =无显著性差异)与实验组相似。所有实验组标本均显示出不同程度的近端小管损伤,表现为细胞空泡化、脱屑、管腔刷状缘丧失,且常伴有凝固性坏死。(摘要截短于250字)

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