中枢神经系统炎症作为补充因子I缺乏的第一个迹象:一种严重的髓炎,用强化免疫疗法和eculizumab治疗
Valérie Massey1, Cam-Tu Emilie Nguyen1, Tine François1
1From the Immunology and Rheumatology Division (V.M., J.J.D.B., H.D.), Department of Pediatrics, Sainte-Justine University Hospital Center; Allergy and Immunology Division (V.M.), Sacré-Coeur Hospital; Neurology Division (C.-T.E.N.), Department of Pediatrics; Intensive Care Division (T.F., A.B.), Department of Pediatrics; Clinical Department of Laboratory Medicine (A.B.), OPTILAB; and Nephrology Division (A.-L.L.), Department of Pediatrics, Sainte-Justine University Hospital Center, Montreal, Canada.
完全补充因子I (CFI) 缺乏可以导致严重的中枢神经系统 (CNS) 炎症作为最初的症状. 快速补充阻塞疗法,如eculizumab,对于控制急性炎症和预防复发至关重要.
科学领域:
- 免疫学 免疫学 免疫学
- 神经学 神经学
- 遗传学 遗传学 是一个
背景情况:
- 补充因子I (CFI) 缺乏症是一种罕见的自体逆性先天性免疫错误.
- CFI在调节补体系统方面发挥着至关重要的作用,这是先天免疫的关键部分.
- 补体级联的调节失调会导致过度的炎症.
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