Patients with Down syndrome (DS) are characterized by increased susceptibility to autoimmunity and respiratory tract infections that are suggestive of humoral immunity impairment. Here, we sought to determine the follicular helper (Tfh) and follicular regulatory (Tfr) T cell profile in the blood of children with DS. Blood was collected from 24 children with DS, nine of which had autoimmune diseases. Children with DS showed skewed Tfh differentiation towards the CXCR3+ phenotype: Tfh1 and Tfh1/17 subsets were increased, while Tfh2 and Tfh17 subsets were reduced. While no differences in the percentage of Tfr cells were seen, the ratio of Tfh1 and CXCR3+PD-1+ subsets to Tfr cells was significantly increased in the affected children. The excessive polarization towards a CXCR3+ phenotype in children with DS suggests that re-calibration of Tfh subset skewing could potentially offer new therapeutic opportunities for these patients.

中文翻译：

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循环滤泡辅助 T 细胞的普遍 CXCR3+ 表型表明唐氏综合症儿童的体液失调。

唐氏综合症 (DS) 患者的特征是对自身免疫和呼吸道感染的易感性增加，这表明体液免疫功能受损。在这里，我们试图确定 DS 儿童血液中的滤泡辅助 (Tfh) 和滤泡调节 (Tfr) T 细胞谱。收集了 24 名患有 DS 的儿童的血液，其中 9 名患有自身免疫性疾病。DS 儿童表现出向 CXCR3+ 表型倾斜的 Tfh 分化：Tfh1 和 Tfh1/17 子集增加，而 Tfh2 和 Tfh17 子集减少。虽然 Tfr 细胞的百分比没有差异，但在受影响的儿童中，Tfh1 和 CXCR3+PD-1+ 亚群与 Tfr 细胞的比例显着增加。