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卡塔格内综合征:六例报告并特别提及体液免疫和细胞免疫

Kartageners syndrome: a report of six cases with special reference to humoral and cellular immunity.

作者信息

Neffen H, Oehling A, Crisci C D

出版信息

Respiration. 1980;40(3):161-7. doi: 10.1159/000194266.

Abstract

4 men and 2 women presenting with Kartagener's syndrome were studied. Humoral and cellular immunity were evaluated by measuring immunoglobulin levels, total complement activity, C3, C4 and C5 levels, lymphocyte subpopulations, lymphoproliferative response to PHA, delayed hypersensitivity skin reactions and histocompatibility antigens. The in vitro tests for cellular and humoral immunity showed normal results, or only transitory alterations. However, the in vivo tests clearly showed a decrease in the delayed response in the skin tests using bacterial and mycotic antigens and PPD. In two siblings the HLA typing showed identical haplotypes. The results are presented taking into account the new physiopathogenetic concepts of Karagener's syndrome with respect to dysfunction of the ciliated columnar epithelium and chemotactic defects of the nonciliated blood cells found in these patients.

摘要

对4名男性和2名患有卡塔格内综合征的女性进行了研究。通过测量免疫球蛋白水平、总补体活性、C3、C4和C5水平、淋巴细胞亚群、对PHA的淋巴细胞增殖反应、迟发型超敏皮肤反应和组织相容性抗原,评估体液免疫和细胞免疫。细胞免疫和体液免疫的体外试验结果正常,或仅有短暂改变。然而,体内试验清楚地显示,在使用细菌和真菌抗原以及PPD进行皮肤试验时,迟发型反应有所降低。在两名同胞中,HLA分型显示单倍型相同。根据卡塔格内综合征关于这些患者中发现的纤毛柱状上皮功能障碍和非纤毛血细胞趋化缺陷的新的生理病理概念呈现结果。

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