儿童和青少年肌无力的针对性治疗。

IF 3.4 3区 医学 Q1 PEDIATRICS
Sithara Ramdas, Teresa Painho, Maria I Vanegas, Dennis T Famili, Ming J Lim, Heinz Jungbluth
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引用次数: 0

摘要

重症肌无力(MG)是一种由抗体介导的神经肌肉接头疾病,多发于儿童和成人。重症肌无力是一种可以治疗的疾病,大多数患者需要通过免疫抑制来控制和/或缓解病情。幼年型重症肌无力(JMG)与成人型重症肌无力相比较为罕见,但由于其基本病理生理学相同,因此治疗策略与成人型重症肌无力相似。直到最近,针对成人肌萎缩症治疗的随机对照试验(RCT)还寥寥无几,而针对儿童肌萎缩症治疗的随机对照试验则一项也没有,治疗策略主要基于专家共识。此外,针对难治性 MG 病例的治疗方案也非常有限,这导致此类患者因疾病负担沉重而长期生活质量低下。最近,有几项 RCT 研究聚焦于新型治疗策略,并取得了潜在的良好疗效,这表明未来几年 MG 的治疗方法将发生改变,MG 患者将获得更有效、起效更快的药物。本文将回顾当前和新的 MG 治疗方法,包括依加替莫德、依库珠单抗、罗扎尼珠单抗、拉武珠单抗和齐鲁珠单抗,重点关注幼年型重症肌无力。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Targeted Treatments for Myasthenia Gravis in Children and Adolescents.

Targeted Treatments for Myasthenia Gravis in Children and Adolescents.

Myasthenia gravis (MG) is an antibody-mediated disorder of the neuromuscular junction affecting children and adults. MG is a treatable condition with most patients requiring immunosuppression for disease control and/or remission. Juvenile myasthenia gravis (JMG) is rare in comparison with adult-onset MG but given the same underlying pathophysiology, treatment strategies are similar to those in adults. Until recently, there were only a few randomised controlled trials (RCTs) for MG treatments in adults and none in children, and management strategies were primarily based on expert consensus. In addition, treatment options for refractory MG cases have been severely limited, resulting in poor long-term quality of life in such patients due to the significant disease burden. Recently, there have been several RCTs focussing on novel therapeutic strategies with potentially promising outcomes, suggesting a change in MG management over the coming years and access to more effective and faster-acting drugs for MG patients. This paper will review current and new MG treatments including efgartigimod, eculizumab, rozanolixizumab, ravulizumab, and zilucoplan, with a focus on juvenile myasthenia gravis.

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来源期刊
Pediatric Drugs
Pediatric Drugs PEDIATRICS-PHARMACOLOGY & PHARMACY
CiteScore
7.20
自引率
0.00%
发文量
54
审稿时长
>12 weeks
期刊介绍: Pediatric Drugs promotes the optimization and advancement of all aspects of pharmacotherapy for healthcare professionals interested in pediatric drug therapy (including vaccines). The program of review and original research articles provides healthcare decision makers with clinically applicable knowledge on issues relevant to drug therapy in all areas of neonatology and the care of children and adolescents. The Journal includes: -overviews of contentious or emerging issues. -comprehensive narrative reviews of topics relating to the effective and safe management of drug therapy through all stages of pediatric development. -practical reviews covering optimum drug management of specific clinical situations. -systematic reviews that collate empirical evidence to answer a specific research question, using explicit, systematic methods as outlined by the PRISMA statement. -Adis Drug Reviews of the properties and place in therapy of both newer and established drugs in the pediatric population. -original research articles reporting the results of well-designed studies with a strong link to clinical practice, such as clinical pharmacodynamic and pharmacokinetic studies, clinical trials, meta-analyses, outcomes research, and pharmacoeconomic and pharmacoepidemiological studies. Additional digital features (including animated abstracts, video abstracts, slide decks, audio slides, instructional videos, infographics, podcasts and animations) can be published with articles; these are designed to increase the visibility, readership and educational value of the journal’s content. In addition, articles published in Pediatric Drugs may be accompanied by plain language summaries to assist readers who have some knowledge of, but not in-depth expertise in, the area to understand important medical advances.
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