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泛亚肌萎缩侧索硬化症治疗与研究联盟(PACTALS)肌萎缩侧索硬化症管理指南

Pan-Asian consortium for treatment and research in ALS (PACTALS) guidelines for management of amyotrophic lateral sclerosis.

作者信息

Vucic Steve, Shahrizaila Nortina, Kano Osamu, Menon Parvathi, Agustini Sheila, Kulkantrakorn Kongkiat, Atchayaram Nalini, Lee Yi-Chung, Prado Mario B, Ng Kay W P, Chai Josiah, Son Bugyeong, Talman Paul, Nghia Hoang Tien Trong, Tuan Le Tu Quoc, Shibuya Kazumoto, Izumi Yuishin, Atsuta Naoki, Henderson Robert D, Cui Liying, Liu Mingsheng, Ohnmar Ohnmar, Rabani Remli, Hong Yoon-Ho, Sung Jung-Joon, Fan Dongsheng, Raykar Veena, Kuwabara Satoshi, Kim Seung Hyun, Sobue Gen, Kiernan Matthew C

机构信息

Brain and Nerve Research Centre, The University of Sydney, Sydney, Australia.

Department of Medicine, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia.

出版信息

Lancet Reg Health West Pac. 2025 Sep 16;62:101684. doi: 10.1016/j.lanwpc.2025.101684. eCollection 2025 Sep.

Abstract

The Pan-Asian Consortium for Treatment and Research in ALS (PACTALS) guidelines were developed for the management of amyotrophic lateral sclerosis (ALS) patients living in the Asia-Pacific countries, taking into consideration the ethnic, racial and economic diversity of the region. The majority of patients reside in low-income (limited-resource setting) and middle-income countries. Grading of Recommendations Assessment, Development, and Evaluation (GRADE) methodology was utilised for development of the PACTALS management guidelines. Nine broad research questions, divided into sections, were addressed. Evidence was derived from existing Cochrane reviews, systematic reviews, meta-analysis, and randomized controlled trials (RCT) along with consensus when evidence was limited. Recommendations were provided for diagnostic pathways, use of disease modifying therapies, appropriateness of multidisciplinary care models, management of respiratory dysfunction, communication and nutrition, addressing symptoms that affect the quality of life, managing cognitive, behavioural and emotional symptoms as well as appropriate implementation of palliative care services and addressing end-of-life issues. The PACTALS guidelines provide a much-needed framework for the management of ALS patients living in the Asia-Pacific region. The management guidelines will be updated as the treatment landscape evolves and evidence of novel management approaches becomes available.

摘要

泛亚肌萎缩侧索硬化症治疗与研究联盟(PACTALS)指南是为生活在亚太国家的肌萎缩侧索硬化症(ALS)患者的管理而制定的,同时考虑到该地区的种族、民族和经济多样性。大多数患者居住在低收入(资源有限环境)和中等收入国家。推荐分级评估、制定与评价(GRADE)方法被用于制定PACTALS管理指南。解决了九个广泛的研究问题,并分为不同部分。证据来源于现有的Cochrane综述、系统评价、荟萃分析和随机对照试验(RCT),在证据有限时还包括共识。针对诊断途径、疾病修饰疗法的使用、多学科护理模式的适宜性、呼吸功能障碍的管理、沟通与营养、处理影响生活质量的症状、管理认知、行为和情绪症状以及适当实施姑息治疗服务和解决临终问题提供了建议。PACTALS指南为亚太地区ALS患者的管理提供了急需的框架。随着治疗格局的演变和新管理方法证据的出现,管理指南将不断更新。

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