Inflammation and lipoperoxidation in mucopolysaccharidoses type II patients at diagnosis and post-hematopoietic stem cell transplantation.

IF 2.5 3区 医学 Q2 MEDICAL LABORATORY TECHNOLOGY
Camila Aguilar Delgado, Franciele Fátima Lopes, Jéssica Lamberty Faverzani, Graziela Schmitt Ribas, Desirèe Padilha Marchetti, Carolina Fischinger Moura de Souza, Roberto Giugliani, Guilherme Baldo, Carmen Regla Vargas
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引用次数: 0

Abstract

Introduction: Mucopolysaccharidosis type II (MPS II) is caused by deficiency of the enzyme iduronate-2-sulfatase; one possible therapy for MPS II is hematopoietic stem cell transplantation (HSCT). It is established that there is excessive production of reactive species in MPS II patients, which can trigger several processes, such as the inflammatory cascade.

Objectives: Our aim was to outline an inflammatory profile and lipoperoxidation of MPS II patients for a better understanding of disease and possible benefits that HSCT can bring in these processes.

Materials and methods: We investigate oxidative damage to lipids by 15-F2t-isoprostane urinary concentrations and plasma pro-and anti-inflammatory cytokine concentrations in MPS II patients at diagnosis, MPS II post-HSCT patients, and controls.

Results: Interleukin (IL)-1β and IL-17a concentrations were significantly increased and a tendency toward increased IL-6 production in the diagnosis group was verified. We found significant decrease in IL-4 and increase in 15-F2t-isoprostane concentrations in the diagnosis group, while IL-1β, IL-6, IL-17a and 15-F2t-isoprostane concentrations were similar between control and post-HSCT groups.

Conclusions: Our study demonstrated that MPS II patients at diagnosis are in a pro-inflammatory state, bringing a novel result showing increased production of IL-17a, an osteoclastogenic cytokine, as well as demonstrating that these patients have oxidative damage to lipids. Furthermore, evidence suggests that HSCT can reduce inflammation and lipoperoxidation in MPS II patients.

黏多醣症 II 型患者在诊断时和造血干细胞移植后的炎症和脂肪过氧化反应。
简介II 型黏多醣症(MPS II)是由于缺乏iduronate-2-sulfatase酶而引起的;造血干细胞移植(HSCT)是治疗MPS II的一种可能方法。目前已证实,MPS II 患者体内会产生过多的活性物质,从而引发多种过程,如炎症级联反应:我们的目的是概述 MPS II 患者的炎症特征和脂质过氧化反应,以便更好地了解疾病以及造血干细胞移植在这些过程中可能带来的益处:我们通过15-F2t-异前列腺烷尿浓度和血浆促炎和抗炎细胞因子浓度调查了MPS II患者诊断时、MPS II造血干细胞移植后和对照组的脂质氧化损伤情况:结果:白细胞介素(IL)-1β和IL-17a的浓度明显升高,而且确诊组中IL-6的产生有增加的趋势。我们发现诊断组的 IL-4 浓度明显下降,15-F2t-异前列腺烷浓度上升,而对照组和 HSCT 术后组的 IL-1β、IL-6、IL-17a 和 15-F2t- 异前列腺烷浓度相似:我们的研究表明,MPS II 患者在确诊时处于促炎症状态,这带来了一个新的结果,即破骨细胞生成细胞因子 IL-17a 的生成增加,同时还表明这些患者的脂质受到氧化损伤。此外,有证据表明造血干细胞移植可以减轻 MPS II 患者的炎症和脂质过氧化反应。
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来源期刊
Clinical biochemistry
Clinical biochemistry 医学-医学实验技术
CiteScore
5.10
自引率
0.00%
发文量
151
审稿时长
25 days
期刊介绍: Clinical Biochemistry publishes articles relating to clinical chemistry, molecular biology and genetics, therapeutic drug monitoring and toxicology, laboratory immunology and laboratory medicine in general, with the focus on analytical and clinical investigation of laboratory tests in humans used for diagnosis, prognosis, treatment and therapy, and monitoring of disease.
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