Baylor College of Medicine/Texas Children's Hospital, Houston, Texas, USA.
Children's National Health System, Washington, District of Columbia, USA.
Ann Clin Transl Neurol. 2020 Dec;7(12):2467-2474. doi: 10.1002/acn3.51229. Epub 2020 Dec 4.
Febrile-infection related epilepsy syndrome (FIRES) is a devastating neurological condition characterized by a febrile illness preceding new onset refractory status epilepticus (NORSE). Increasing evidence suggests innate immune dysfunction as a potential pathological mechanism. We report an international retrospective cohort of 25 children treated with anakinra, a recombinant interleukin-1 receptor antagonist, as an immunomodulator for FIRES. Anakinra was potentially safe with only one child discontinuing therapy due to infection. Earlier anakinra initiation was associated with shorter duration of mechanical ventilation, ICU and hospital length of stay. Our retrospective data lay the groundwork for prospective consensus-driven cohort studies of anakinra in FIRES.
发热感染相关癫痫综合征（FIRES）是一种破坏性的神经疾病，其特征是在新发难治性癫痫持续状态（NORSE）之前有发热性疾病。越来越多的证据表明固有免疫功能障碍是一种潜在的病理机制。我们报告了一项国际回顾性队列研究，该研究纳入了 25 名接受阿那白滞素治疗的儿童，阿那白滞素是一种重组白细胞介素-1 受体拮抗剂，作为 FIRES 的免疫调节剂。阿那白滞素的潜在安全性良好，仅有 1 名患儿因感染而停止治疗。早期使用阿那白滞素与机械通气、重症监护病房和住院时间的缩短有关。我们的回顾性数据为 FIRES 中阿那白滞素的前瞻性共识驱动队列研究奠定了基础。
