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Inflammatory involvement into phototoxic reaction in erythropoietic protoporphyria (EPP) patients.
Immunologic Research ( IF 3.3 ) Pub Date : 2019-10-01 , DOI: 10.1007/s12026-019-09097-5
Francesca Granata 1 , Lorena Duca 1 , Giovanna Graziadei 1 , Valentina Brancaleoni 1 , Pasquale Missineo 2 , Giacomo De Luca 1 , Silvia Fustinoni 3 , Elena Di Pierro 1
Affiliation  

Phototoxic reaction is a known feature of EPP at least in part triggered by the oxidative status, complement system activation, and mast cell response. The aim of this study was to verify some aspects involved in phototoxic reaction during a season. The complement system was evaluated by C3 assay, alternative pathway by factor-B, and classical pathway by C1q; oxidative status was tested with malondialdehyde (MDA) and mast cell by IL-10 assay. The serum samples were collected in winter and summer from 19 EPP patients and 13 controls. The reaction to sun exposure within each group was monitored without any invasive treatment. In summer, C3 and factor B were higher in patients than in controls (p = 0.002 and < 0.0001 respectively), while no change was detected for C1q. The oxidative stress was increased in summer in comparison with the control group (p = 0.04), and IL-10 an assay was normal in both seasons. The correlation between the C3 and factor-B in summer was significant. This study shows that the phototoxic reaction is not limited to the dermis but can also exert a systemic response, which could affect the general health of a patient. The knowledge of the pathophysiology of phototoxic reaction is essential for identifying new disease markers useful for improving clinical studies of known and future drugs.

中文翻译:

红细胞生成性原卟啉症(EPP)患者的炎症参与光毒性反应。

光毒反应是EPP的已知特征,至少部分地由氧化状态,补体系统激活和肥大细胞反应触发。这项研究的目的是验证一个季节中涉及光毒性反应的某些方面。通过C3分析评估补体系统,通过B因子评估替代途径,并通过C1q评估经典途径。氧化状态用丙二醛(MDA)和肥大细胞通过IL-10测定。在冬季和夏季从19名EPP患者和13名对照中收集血清样本。在没有任何侵入性治疗的情况下,监测每组中对日光照射的反应。在夏季,患者中的C3和B因子高于对照组(分别为p = 0.002和<0.0001),而C1q没有变化。与对照组相比,夏季的氧化应激增加(p = 0.04),IL-10检测在两个季节均正常。夏季C3和B因子之间的相关性很显着。这项研究表明,光毒性反应不仅限于真皮,还可以产生全身反应,这可能会影响患者的整体健康。光毒性反应的病理生理学知识对于鉴定可用于改善已知和未来药物临床研究的新疾病标志物至关重要。
更新日期:2019-11-01
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