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普通可变型免疫缺陷:一项家族研究及西咪替丁治疗试验

Common variable immunodeficiency: a family study and therapeutic trial with cimetidine.

作者信息

Segal R, Dayan M, Epstein N, Zecler E, Peller S, Michalevitch R, Brautbar C, Moses E

机构信息

Department of Medicine, Vancouver General Hospital, University of British Columbia, Canada.

出版信息

J Allergy Clin Immunol. 1989 Nov;84(5 Pt 1):753-61. doi: 10.1016/0091-6749(89)90305-9.

Abstract

Various immunologic parameters were tested in a family of two patients with common variable immunodeficiency. Both patients had low serum immunoglobulin levels, low peripheral B cell and T4 subclass of lymphocytes, and reversed T4/T8 ratio. One of the patients had excessive suppressor activity. Two of the asymptomatic members of the family (the father and one brother) had also low T4/T8 ratio that was not associated with excessive suppressor activity. No linkage between the disease inheritance to HLA could be observed. A study of the T cell helper activity to an antigen, the response to which is regulated by an HLA-linked gene, suggested a defect in the immune response of the two patients and their asymptomatic brother with immunologic disorders. Treatment with cimetidine of the patient with excessive suppressor activity led to an improvement in his clinical state, reduction in suppressor activity, temporary effect on his proliferative response capacity to mitogens, and an increase in the antigen-specific helper activity.

摘要

对一个患有常见可变免疫缺陷的两名患者的家庭进行了各种免疫学参数检测。两名患者血清免疫球蛋白水平均低,外周血B细胞和淋巴细胞T4亚类低,且T4/T8比值倒置。其中一名患者具有过度的抑制活性。该家庭的两名无症状成员(父亲和一个兄弟)T4/T8比值也低,但与过度抑制活性无关。未观察到疾病遗传与HLA之间的连锁关系。对一种抗原的T细胞辅助活性的研究表明,该抗原的反应受HLA连锁基因调控,提示两名患者及其有免疫紊乱的无症状兄弟的免疫反应存在缺陷。对具有过度抑制活性的患者用西咪替丁治疗后,其临床状态有所改善,抑制活性降低,对其对有丝分裂原的增殖反应能力有暂时影响,且抗原特异性辅助活性增加。

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