1型强直性肌营养不良症成人患者基于共识的护理建议。
Consensus-based care recommendations for adults with myotonic dystrophy type 1.
作者信息
Ashizawa Tetsuo, Gagnon Cynthia, Groh William J, Gutmann Laurie, Johnson Nicholas E, Meola Giovanni, Moxley Richard, Pandya Shree, Rogers Mark T, Simpson Ericka, Angeard Nathalie, Bassez Guillaume, Berggren Kiera N, Bhakta Deepak, Bozzali Marco, Broderick Ann, Byrne Janice L B, Campbell Craig, Cup Edith, Day John W, De Mattia Elisa, Duboc Denis, Duong Tina, Eichinger Katy, Ekstrom Anne-Berit, van Engelen Baziel, Esparis Belen, Eymard Bruno, Ferschl Marla, Gadalla Shahinaz M, Gallais Benjamin, Goodglick Todd, Heatwole Chad, Hilbert James, Holland Venessa, Kierkegaard Marie, Koopman Wilma J, Lane Kari, Maas Daphne, Mankodi Ami, Mathews Katherine D, Monckton Darren G, Moser David, Nazarian Saman, Nguyen Linda, Nopoulos Peg, Petty Richard, Phetteplace Janel, Puymirat Jack, Raman Subha, Richer Louis, Roma Elisabetta, Sampson Jacinda, Sansone Valeria, Schoser Benedikt, Sterling Laurie, Statland Jeffrey, Subramony S H, Tian Cuixia, Trujillo Careniña, Tomaselli Gordon, Turner Chris, Venance Shannon, Verma Aparajitha, White Molly, Winblad Stefan
出版信息
Neurol Clin Pract. 2018 Dec;8(6):507-520. doi: 10.1212/CPJ.0000000000000531.
PURPOSE OF REVIEW
Myotonic dystrophy type 1 (DM1) is a severe, progressive genetic disease that affects between 1 in 3,000 and 8,000 individuals globally. No evidence-based guideline exists to inform the care of these patients, and most do not have access to multidisciplinary care centers staffed by experienced professionals, creating a clinical care deficit.
RECENT FINDINGS
The Myotonic Dystrophy Foundation (MDF) recruited 66 international clinicians experienced in DM1 patient care to develop consensus-based care recommendations. MDF created a 2-step methodology for the project using elements of the Single Text Procedure and the Nominal Group Technique. The process generated a 4-page Quick Reference Guide and a comprehensive, 55-page document that provides clinical care recommendations for 19 discrete body systems and/or care considerations.
SUMMARY
The resulting recommendations are intended to help standardize and elevate care for this patient population and reduce variability in clinical trial and study environments.
综述目的
1型强直性肌营养不良症（DM1）是一种严重的进行性遗传病，全球每3000至8000人中就有1人受其影响。目前尚无基于证据的指南为这些患者的护理提供指导，而且大多数患者无法获得由经验丰富的专业人员组成的多学科护理中心的服务，这造成了临床护理的不足。
最新发现
强直性肌营养不良症基金会（MDF）招募了66名在DM1患者护理方面经验丰富的国际临床医生，以制定基于共识的护理建议。MDF使用单一文本程序和名义群体技术的要素为该项目创建了一个两步法。该过程产生了一份4页的快速参考指南和一份全面的55页文件，为19个不同的身体系统提供临床护理建议和/或护理注意事项。
总结
由此产生的建议旨在帮助规范和提升对这一患者群体的护理，并减少临床试验和研究环境中的差异。
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