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首页> 外文期刊>Clinical immunology: The official journal of the Clinical Immunology Society >B-cell activating factor (BAFF) is elevated in Chronic Granulomatous Disease
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B-cell activating factor (BAFF) is elevated in Chronic Granulomatous Disease

机译：慢性肉芽肿病中B细胞活化因子（BAFF）升高

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摘要

Chronic Granulomatous Disease (CGD) is an inherited defect in superoxide production leading to life-threatening infections, granulomas, and, possibly, abnormal immunoglobulin concentrations. We investigated whether factors controlling antibody production, such as B-cell activating factor (BAFF), were altered in CGD. CGD subjects had significantly increased mean (2.3-fold, p. <. 0.0001) plasma concentrations of BAFF compared to healthy donors. Patients on IFN-γ treatment had significantly higher BAFF concentrations compared with CGD patients not taking IFN-γ (1.6-fold, p. <. 0.005). Leukocytes from CGD subjects produced normal amounts of BAFF in response to IFN-γ or G-CSF in vitro. Expression of BAFF-R and TACI was significantly reduced on CGD B cells. Elevated BAFF in CGD correlated with CRP ( R= 0.44), ESR ( R= 0.49), and IgM ( R= 0.47) and increased rapidly in healthy subjects following intravenous endotoxin administration. These findings suggest that elevated BAFF in CGD subjects and healthy donors is a consequence of acute and chronic inflammation.
机译：慢性肉芽肿性疾病（CGD）是超氧化物生成的遗传缺陷，导致威胁生命的感染，肉芽肿以及可能的异常免疫球蛋白浓度。我们调查了控制抗体产生的因素，例如B细胞活化因子（BAFF），在CGD中是否发生了改变。与健康供体相比，CGD受试者的平均血浆BAFF浓度显着增加（2.3倍，p。<0.0001）。与未服用IFN-γ的CGD患者相比，接受IFN-γ治疗的患者的BAFF浓度明显更高（1.6倍，p。<0.005）。来自CGD受试者的白细胞在体外对IFN-γ或G-CSF产生正常量的BAFF。在CGD B细胞上，BAFF-R和TACI的表达明显降低。 CGD中BAFF升高与CRP（R = 0.44），ESR（R = 0.49）和IgM（R = 0.47）相关，并且在静脉内给予内毒素后在健康受试者中迅速升高。这些发现表明，CGD受试者和健康供体的BAFF升高是急性和慢性炎症的结果。

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