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有临床症状提示可能存在原发性免疫缺陷的儿童的胸腺输出变化。

Thymic output changes in children with clinical findings signaling a probable primary immunodeficiency.

作者信息

Karaca Neslihan, Azarsız Elif, Akarcan Sanem Eren, Aksu Güzide, Kütükçüler Necil

机构信息

Departments of Pediatric Immunology, Ege University Faculty of Medicine, İzmir, Turkey.

Departments of Biochemistry, Ege University Faculty of Medicine, İzmir, Turkey.

出版信息

Turk J Pediatr. 2019;61(6):885-894. doi: 10.24953/turkjped.2019.06.010.

Abstract

Karaca N, Azarsız E, Akarcan SE, Aksu G, Kütükçüler N. Thymic output changes in children with clinical findings signaling a probable primary immunodeficiency. Turk J Pediatr 2019; 61: 885-894. Thymic maturation evaluation is inevitable for patients with clinical and laboratory findings for a primary immunodeficiency, as the T cellimmunodeficiencies are the most severe type. In this study, we aimed to show the usage of T cell surface molecule CD31 for the evaluation of thymic output in patients (n: 66) with a large spectrum of findings signing a probable primary immunodeficiency. Besides the classical clinical and laboratory approach for these patients, T cell subpopulations as naive, memory, recent thymic emigrant cells were also investigated. The humoral immunodeficiency (34.8%), combined immunodeficiency (34.8%) and cardiopathy (7.6%) were the most frequent diagnosis groups. CD4+CD45RA+ naive T-cells percentages (p: 0.011) and absolute counts (p: 0.004) and absolute CD4+CD45RA+CD31+ RTE (recent thymic emigrant) cell counts (p: 0.007) were significantly lower in combined immunodeficiency group. Naive T-cells (p: 0.037) and RTE cells (p: 0.032) were also lower in patients who had cardiac surgery in the past. In conclusion, flow cytometric CD31+thymic naive RTE cell evaluation may provide rapid clinical information especially on T-cell immune dysfunction and CD4+CD45RA+CD31+ RTE cells may be used as an alternative to TRECs in the diagnosis of combined immunodeficiencies.

摘要

卡拉卡 N、阿扎尔西兹 E、阿克卡尔坎 SE、阿克苏 G、库图克彻勒 N。有提示可能存在原发性免疫缺陷临床症状的儿童胸腺输出变化。《土耳其儿科学杂志》2019 年;61: 885 - 894。由于 T 细胞免疫缺陷是最严重的类型,对于有原发性免疫缺陷临床和实验室检查结果的患者,胸腺成熟评估是必不可少的。在本研究中,我们旨在展示 T 细胞表面分子“CD31”在评估有广泛提示可能存在原发性免疫缺陷症状的患者(n = 66)胸腺输出中的应用。除了对这些患者采用经典的临床和实验室方法外,还对幼稚、记忆、近期胸腺迁出细胞等 T 细胞亚群进行了研究。体液免疫缺陷(34.8%)、联合免疫缺陷(34.8%)和心脏病(7.6%)是最常见的诊断组。联合免疫缺陷组中 CD4 + CD45RA + 幼稚 T 细胞百分比(p = 0.011)、绝对计数(p = 0.004)以及绝对 CD4 + CD45RA + CD31 + RTE(近期胸腺迁出)细胞计数(p = 0.007)显著更低。既往接受心脏手术的患者中幼稚 T 细胞(p = 0.037)和 RTE 细胞(p = 0.032)也更低。总之,流式细胞术检测 CD31 + 胸腺幼稚 RTE 细胞评估可能提供快速临床信息,尤其是关于 T 细胞免疫功能障碍方面,并且 CD4 + CD45RA + CD31 + RTE 细胞可作为联合免疫缺陷诊断中 TREC 的替代指标。

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