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低丙种球蛋白血症的肺部表现。

Pulmonary manifestations of hypogammaglobulinaemia.

作者信息

Dukes R J, Rosenow E C, Hermans P E

出版信息

Thorax. 1978 Oct;33(5):603-7. doi: 10.1136/thx.33.5.603.

DOI:10.1136/thx.33.5.603
PMID:725827
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC470942/
Abstract

Fifty-five patients with late-onset idiopathic immunoglobulin deficiency were studied and upper or lower respiratory tract infections were encountered in about 90%. Cylindrical bronchiectasis was shown in all of the 21 patients in whom bronchograms were done. A thymoma was found in four patients. Three patients had diffuse interstitial pulmonary disease--two with proved and one with presumed lymphocytic interstitial pneumonitis. Five patients had no evidence of pulmonary disease, including two patients with long-standing late-onset immunoglobulin deficiency who had essentially no serum immunoglobulins. This small subgroup of patients with immunoglobulin dificiency without severe pulmonary infections cannot be explained in the context of current understanding of immunoglobulin deficiency. Thirty-two patients were followed up for long enough for the response to treatment to be assessed.

摘要

对55例迟发性特发性免疫球蛋白缺乏症患者进行了研究,约90%的患者出现上呼吸道或下呼吸道感染。在接受支气管造影的21例患者中均显示有圆柱状支气管扩张。4例患者发现有胸腺瘤。3例患者有弥漫性间质性肺疾病——2例经证实,1例推测为淋巴细胞间质性肺炎。5例患者无肺部疾病证据,其中2例患有长期迟发性免疫球蛋白缺乏症,基本没有血清免疫球蛋白。这一小部分免疫球蛋白缺乏但无严重肺部感染的患者,根据目前对免疫球蛋白缺乏的认识无法解释。对32例患者进行了足够长时间的随访,以评估治疗反应。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eb6e/470942/cb195a3f0d1c/thorax00161-0062-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eb6e/470942/3b54ddde46e7/thorax00161-0061-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eb6e/470942/cb195a3f0d1c/thorax00161-0062-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eb6e/470942/3b54ddde46e7/thorax00161-0061-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eb6e/470942/cb195a3f0d1c/thorax00161-0062-a.jpg

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What is Good's syndrome? Immunological abnormalities in patients with thymoma.什么是古德综合征?胸腺瘤患者的免疫异常。
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“获得性”或常见变异型无丙种球蛋白血症的异质性。
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Diffuse pulmonary lymphoreticular infiltrations associated with dysproteinemia.与蛋白异常血症相关的弥漫性肺淋巴网状浸润
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Role of suppressor T cells in pathogenesis of common variable hypogammaglobulinaemia.抑制性T细胞在常见变异型低丙种球蛋白血症发病机制中的作用。
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