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韦格纳肉芽肿病:三例病例综述

WEGENER'S GRANULOMATOSIS: A REVIEW OF THREE CASES.

作者信息

FISHER J H

出版信息

Can Med Assoc J. 1964 Jan 4;90(1):10-4.

Abstract

Clinical and autopsy features in three cases of Wegener's granulomatosis are presented. The disease is characterized by the triad of necrotizing granulomatous lesions of the respiratory tract, segmental necrotizing angiitis of arteries and veins, and necrotizing glomerulitis commonly terminating in uremia. Correct antemortem diagnosis was made in two of the three cases-in one by recognition of the characteristic triad and in the other by a biopsy from the antrum. Pathological lesions were chiefly in respiratory tract, spleen, kidneys and blood vessels. Necrotizing granulomatous lesions were present in the lungs. Glomerular lesions were particularly severe and wide-spread in two of the three patients, leading to uremia. Trabeculitis of the spleen, an unusual lesion, was a noticeable feature in all three. Fibrinoid necrosis, with little or no inflammatory infiltration, was the prevailing type of vascular necrosis. The characteristics of the disease process suggest a hypersensitivity mechanism. Similarities to, but significant differences from, polyarteritis nodosa are recognized.

摘要

本文介绍了三例韦格纳肉芽肿病的临床和尸检特征。该病的特点是呼吸道坏死性肉芽肿性病变、动静脉节段性坏死性血管炎以及通常导致尿毒症的坏死性肾小球肾炎三联征。三例中有两例在生前做出了正确诊断,一例是通过识别特征性三联征,另一例是通过上颌窦活检。病理病变主要见于呼吸道、脾脏、肾脏和血管。肺部存在坏死性肉芽肿性病变。在三例患者中有两例肾小球病变特别严重且广泛,导致了尿毒症。脾脏小梁炎是一种不常见的病变,在所有三例中都是一个显著特征。纤维蛋白样坏死,炎症浸润很少或没有,是血管坏死的主要类型。疾病过程的特征提示了一种超敏反应机制。认识到与结节性多动脉炎有相似之处,但也有显著差异。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d772/1922313/5b8953879112/canmedaj01029-0011-a.jpg

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