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Downregulation of B regulatory cells and upregulation of T helper 1 cells in children with Gaucher disease undergoing enzyme replacement therapy.
Immunologic Research ( IF 4.4 ) Pub Date : 2020-06-10 , DOI: 10.1007/s12026-020-09129-5
Asmaa M Zahran 1 , Mervat A M Youssef 2 , Engy Adel Shafik 1 , Zeinab Albadry M Zahran 3 , Omnia El-Badawy 4 , Amir M Abo Elgheet 2 , Khalid I Elsayh 2
Affiliation  

Gaucher disease (GD) involves a broad spectrum of immunological cells, including T helper (Th) cells and regulatory B cells (Bregs), which function to resolve the immune response and inhibit excessive inflammation. This study aimed to explore T helper cells, B cells, and Bregs in GD children undergoing enzyme replacement therapy (ERT). Our study included 20 GD patients; six patients were categorized as type 1 and 14 as type 3 GD. All patients were on regular ERT. Twenty healthy children were enrolled as controls. All patients and controls were subjected to complete blood analysis, abdominal ultrasound, and flow cytometric detection of T helper cells, B cells, and Bregs. Despite undergoing ERT, CD4+ T helper lymphocytes and Bregs were still significantly lower in patients with GD compared with the controls. Th1 and B cells were more in the patients than in the healthy controls. Lower levels of Bregs were found in type 3, compared with type 1 patients. Increased platelet count was directly associated with increased levels of Bregs and lower levels of B cells. Elevated children’s height was also accompanied by decreasing levels of Th1. Our results propose that ERT in GD is associated with partial improvement in immune status, and long-term ERT might be needed for the restoration of the desired immune response levels. Levels of Bregs and Th1 can be employed for monitoring improvement of immune status in GD patients undergoing ERT.



中文翻译:

接受酶替代疗法治疗的高雪病患儿的B调节细胞下调和T辅助1细胞上调。

高雪氏病(GD)涉及广泛的免疫细胞,包括T辅助(Th)细胞和调节性B细胞(Bregs),它们的功能是解决免疫反应并抑制过度炎症。这项研究旨在探讨接受酶替代疗法(ERT)的GD儿童的T辅助细胞,B细胞和Bregs。我们的研究包括20名GD患者。六例患者被分为1型和14型为3型GD。所有患者均接受常规ERT治疗。二十名健康儿童作为对照。所有患者和对照均进行了全血分析,腹部超声检查以及T辅助细胞,B细胞和Bregs的流式细胞仪检测。尽管经历了ERT,GD患者的CD4 + T辅助淋巴细胞和Bregs仍显着低于对照组。患者中的Th1和B细胞比健康对照者多。与1型患者相比,3型患者的Bregs水平较低。血小板计数增加与Bregs水平升高和B细胞水平降低直接相关。儿童身高升高也伴随着Th1水平的降低。我们的结果表明,GD中的ERT与免疫状态的部分改善有关,可能需要长期的ERT才能恢复所需的免疫应答水平。Bregs和Th1的水平可用于监测接受ERT的GD患者的免疫状况。儿童身高升高也伴随着Th1水平的降低。我们的结果表明,GD中的ERT与免疫状态的部分改善有关,可能需要长期的ERT才能恢复所需的免疫应答水平。Bregs和Th1的水平可用于监测接受ERT的GD患者的免疫状况。儿童身高升高也伴随着Th1水平的降低。我们的研究结果表明,GD中的ERT与免疫状态的部分改善有关,可能需要长期的ERT才能恢复所需的免疫应答水平。Bregs和Th1的水平可用于监测接受ERT的GD患者的免疫状况。

更新日期:2020-06-10
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