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肺泡蛋白沉积症患者支气管肺泡灌洗流出液的成分

Composition of bronchoalveolar lavage effluents from patients with pulmonary alveolar proteinosis.

作者信息

Hook G E, Bell D Y, Gilmore L B, Nadeau D, Reasor M J, Talley F A

出版信息

Lab Invest. 1978 Oct;39(4):342-57.

PMID:212641
Abstract

The lungs of patients with pulmonary alveolar proteinosis were lavaged with saline. The lavage effluents were examined for the presence of unusual components which might be considered characteristic of the disease. Soluble proteins were resolved by electrophoresis on polyacrylamide linear gradient (2.5 to 27 per cent acrylamide) gel slabs and compared with proteins found in the patients' sera and lavage effluents from nondiseased human lungs. Many proteins in lavage effluents from both diseased and nondiseased lungs appeared similar to those found in serum. Several proteins from diseased lungs were not present in serum nor lavage effluents from nondiseased lungs. A number of proteins present in lavage effluents from nondiseased lungs appeared to be absent from lungs with alveolar proteinosis. Particulate components of lavage effluents from diseased and normal lungs were examined and compared using the electron microscope. Many particulate components including degenerate and disintegrating cells, ultrastructurally abnormal alveolar macrophages, and myelin-like structures were present in lavage effluents from diseased lungs but absent from normal lungs. Much of the particulate material resembled cell debris. The insoluble accumulations present in the pulmonary alveoli and airways of patients with alveolar proteinosis do not come from serum leakage nor from hypersecretion of materials normally found in these regions of the lungs. The major insoluble components appear to arise from cellular disintegration and the extracellular formation of myelin-like structures.

摘要

对肺泡蛋白沉积症患者的肺部进行生理盐水灌洗。检查灌洗流出液中是否存在可能被视为该疾病特征的异常成分。可溶性蛋白质通过在聚丙烯酰胺线性梯度(2.5%至27%丙烯酰胺)凝胶板上进行电泳分离,并与患者血清以及非患病人类肺部灌洗流出液中的蛋白质进行比较。患病和非患病肺部灌洗流出液中的许多蛋白质与血清中的蛋白质相似。患病肺部的几种蛋白质在血清和非患病肺部的灌洗流出液中均未出现。非患病肺部灌洗流出液中存在的一些蛋白质在肺泡蛋白沉积症患者的肺部中似乎不存在。使用电子显微镜对患病和正常肺部灌洗流出液的颗粒成分进行检查和比较。患病肺部的灌洗流出液中存在许多颗粒成分,包括退化和正在解体的细胞、超微结构异常的肺泡巨噬细胞以及髓磷脂样结构,而正常肺部中则没有。许多颗粒物质类似于细胞碎片。肺泡蛋白沉积症患者肺泡和气道中存在的不溶性积聚物并非来自血清渗漏,也不是来自肺部这些区域中正常存在物质的过度分泌。主要的不溶性成分似乎源于细胞解体以及髓磷脂样结构的细胞外形成。

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