目的:根据最佳证据和经验,为患有NORSE/FIRES的成人和儿科患者的管理制定基于共识的建议。
方法:遵循德尔菲法。成立了一个由9名专家组成的促进小组,定义范围的人,用户和建议的建议。在对当前文献进行回顾之后,关于诊断的建议声明,产生了治疗和研究方向,然后由该领域的48位专家组成的小组以1(强烈不同意)到9(强烈同意)的比例投票。一致认为,如果中位数得分大于或等于7,则陈述是适当的,如果中位数得分小于或等于3,则陈述是不适当的。证据分析被映射到Delphi调查中包含的每个陈述的结果。
结果:总体而言,85项建议声明达成共识。建议分为五个部分:1)疾病特征,2)诊断测试和取样,3)急性治疗,4)急性期后治疗,5)研究,登记册和未来的方向在NORSE/FIRES。本文概述了所有85种陈述的详细结果和讨论。研究结果和实际流程图的相应摘要在配套文章中介绍。
结论:此详细分析提供了有关支持证据和当前文献中与NORSE/FIRES相关的专家共识声明相关的空白的见解。这一共识产生的建议可以作为诊断的指南,评估,以及对NORSE/FIRES患者的管理,以及对未来研究的规划。
OBJECTIVE: To develop
consensus-based recommendations for the management of adult and paediatric patients with NORSE/FIRES based on best evidence and experience.
METHODS: The Delphi methodology was followed. A facilitator group of 9 experts was established, who defined the scope, users and suggestions for recommendations. Following a review of the current literature, recommendation statements concerning diagnosis, treatment and research directions were generated which were then voted on a scale of 1 (strongly disagree) to 9 (strongly agree) by a panel of 48 experts in the field.
Consensus that a statement was appropriate was reached if the median score was greater or equal to 7, and inappropriate if the median score was less than or equal to 3. The analysis of evidence was mapped to the results of each statement included in the Delphi survey.
RESULTS: Overall, 85 recommendation statements achieved
consensus. The recommendations are divided into five sections: 1) disease characteristics, 2) diagnostic testing and sampling, 3) acute treatment, 4) treatment in the post-acute phase, and 5) research, registries and future directions in NORSE/FIRES. The detailed results and discussion of all 85 statements are outlined herein. A corresponding summary of findings and practical flowsheets are presented in a companion article.
CONCLUSIONS: This detailed analysis offers insight into the supporting evidence and the current gaps in the literature that are associated with expert
consensus statements related to NORSE/FIRES. The recommendations generated by this
consensus can be used as a guide for the diagnosis, evaluation, and management of patients with NORSE/FIRES, and for planning of future research.