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[轻链肾病]

[Light-chain nephropathy].

作者信息

Iványi B, Nagy J, Varga G, Búzás E

机构信息

II. Belgyógyászati Klinika, Szent-Györgyi Albert Orvostudományi Egyetem Pathologiai Intézet, Szeged.

出版信息

Orv Hetil. 1990 Aug 19;131(33):1791-5.

PMID:2119026
Abstract

The authors deal with the clinicopathology of the renal, alterations in light-chain disease in connection with 6 cases. The disease was recognized by the monotype (in 5 cases kappa, in 1 case lambda) immunoreactivity of the light-chain paraprotein deposited in the basal membranes of the renal tissue. Electron microscopic examinations proved the fine-granulated, electrodense character of the paraprotein. Multiple myeloma was found in 3 cases and plasma cell dyscrasia of non-tumorous characteristic in 3 cases in the background of the deposition. The renal involvement appeared clinically in the picture of proteinuria without nephrosis syndrome and in progressing azotemia. Chronic renal insufficiency developed during some months in 5 patients. Morphologically renal impairment manifested in interstitial fibrosis, tubular atrophy and ateriolar hyalinosis was seen. These were associated with different glomerular alterations, for instance in 3 cases with nodular glomerulosclerosis. In 1 patient with plasma cell dyscrasia of non-tumorous characteristic nodular glomerulosclerosis and semilunar formation was observed in 56% of the glomeruli. In an other patient with myeloma the simultaneous existence of cylinder nephropathy and light-chain nephropathy was demonstrated. Both observations are unusual phenomena in plasma cell dyscrasia.

摘要

作者探讨了6例与轻链病相关的肾脏临床病理学改变。通过沉积于肾组织基底膜的轻链副蛋白的单型免疫反应性(5例为kappa型,1例为lambda型)确诊该病。电子显微镜检查证实了副蛋白的细颗粒状、电子致密特征。在沉积的背景下,3例发现多发性骨髓瘤,3例发现非肿瘤性特征的浆细胞异常增生。肾脏受累在临床上表现为无肾病综合征的蛋白尿和进行性氮质血症。5例患者在数月内发展为慢性肾功能不全。形态学上,肾脏损害表现为间质纤维化、肾小管萎缩和小动脉玻璃样变。这些与不同的肾小球改变相关,例如3例出现结节性肾小球硬化。在1例具有非肿瘤性特征的浆细胞异常增生患者中,56%的肾小球观察到结节性肾小球硬化和半月形形成。在另1例骨髓瘤患者中,证实同时存在管型肾病和轻链肾病。这两种观察结果在浆细胞异常增生中均为不寻常现象。

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