中文摘要：

目的 探讨BLyS／April在川崎病（KD）免疫发病机制中的作用。方法 急性期KD患儿48例，正常同年龄对照组32名，KD患儿分别于静脉注射丙种球蛋白（IVIG）治疗前后直接取血备检。采用反转录-聚合酶链反应（RT-PCR）及荧光定量PCR检测单核-巨噬细胞（MC）BLyS和April，BLyS／April受体BR3、BCMA、TACI及细胞因子mRNA表达；双抗体夹心酶联免疫吸附试验（ELISA）检测血浆中细胞因子蛋白浓度。结果 ①急性期KD患儿MCBLyS和Aprilm RNA表达明显高于正常同年龄对照组（P〈0．01），经IVIG治疗后表达不同程度下降；②BLyS／April受体BR3、BCMA和TACI基因转录水平显著高于正常同年龄对照组（P〈0．01）；③急性期KD患儿抗体类型转换相关细胞因子白细胞介素（IL）-4、IL-5、IL-6、IL-10、IL-15 mRNA表达水平及蛋白浓度明显高于正常同年龄对照组（P〈0．01）；④KD患儿BLyS／April调控因子干扰素（IFN）-α/γ、IL-10 mRNA表达水平及血浆浓度明显高于正常同年龄对照组（P〈0．01）。结论 急性期KD患儿BLyS／April过表达可能是导致KD免疫功能紊乱的因素之一。

英文摘要：

Objective To investigate the roles of BLyS/April in immunological pathogensis of Kawasaki disease （KD）. Methods Forty-eight children with Kawasaki disease and 32 age-matched healthy children were studied. Reverse-transcription PCR（RT-PCR） and real-time PCR were used to measure the mRNA levels of BLyS/April, receptors for BLyS/April （BR3, BCMA and TACI） and cytokines in patients with KD. The levels of cytokines in plasma were measured by ELISA. Results ① Compared with healthy controls, transcription levels of BLyS/April in MC were significantly up-regulated during acute phase of KD （P〈0.01）, and down-regulated after treated with intravenous immunoglobulin therapy. ② The expression levels of the BLyS/ April receptors BR3, BCMA and TACI mRNA were remarkably increased during acute phase of Kawasaki disease （P〈0.01）, and down-regulated to some extents after treated with intravenous immunoglobulin therapy. ③The mRNA levels and plasma levels of cytokines such as IL-4, IL-5, IL-6, IL-10 and IL-15, which were in- volved in antibody class switching, were found to be significantly higher than those of healthy controls during acute phase of Kawasaki disease （P〈0.01）. ④ The mRNA levels and the plasma levels of IL-10, IFN-α and 1FN-5,, were significantly elevated during acute phase of Kawasaki disease. Conclusion Overexpression of BLyS/April may be one contributing factor to the immunological dysfunction in KD.