Significant inconsistencies in respiratory care provision for Duchenne muscular dystrophy (DMD) are reported across different specialist neuromuscular centres in the UK. The absence of robust clinical evidence and expert consensus is a barrier to the implementation of care recommendations in public healthcare systems as is the need to increase awareness of key aspects of care for those living with DMD. Here, we provide evidenced-based and/or consensus-based best practice for the respiratory care of children and adults living with DMD in the UK, both as part of routine care and in an emergency. Methodology Initiated by an expert working group of UK-based respiratory physicians (including British Thoracic Society (BTS) representatives), neuromuscular clinicians, physiotherapist and patient representatives, draft guidelines were created based on published evidence, current practice and expert opinion. After wider consultation with UK respiratory teams and neuromuscular services, consensus was achieved on these best practice recommendations for respiratory care in DMD. Result The resulting recommendations are presented in the form of a flow chart for assessment and monitoring, with additional guidance and a separate chart setting out key considerations for emergency management. The recommendations have been endorsed by the BTS. Conclusions These guidelines provide practical, reasoned recommendations for all those managing day-to-day and acute respiratory care in children and adults with DMD. The hope is that this will support patients and healthcare professionals in accessing high standards of care across the UK.

中文翻译：

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英国杜氏肌营养不良症呼吸护理指南的制定：临床实践的关键建议

据报道，英国不同的神经肌肉专科中心为杜氏肌营养不良症 (DMD) 提供的呼吸护理服务存在显着不一致。缺乏强有力的临床证据和专家共识是在公共医疗保健系统中实施护理建议的障碍，因为需要提高对 DMD 患者护理关键方面的认识。在这里，我们为英国 DMD 儿童和成人的呼吸护理提供基于证据和/或基于共识的最佳实践，作为常规护理和紧急情况的一部分。方法由英国呼吸科医生（包括英国胸科学会 (BTS) 代表）、神经肌肉临床医生、物理治疗师和患者代表组成的专家工作组发起，根据已发表的证据、当前实践和专家意见制定了指南草案。在与英国呼吸团队和神经肌肉服务部门进行更广泛的磋商后，就 DMD 呼吸护理的最佳实践建议达成了共识。结果 由此产生的建议以评估和监测流程图的形式提出，并附有额外的指导和单独的图表，列出了应急管理的关键考虑因素。这些建议已得到 BTS 的认可。结论 这些指南为所有管理 DMD 儿童和成人日常和急性呼吸护理的人员提供了实用、合理的建议。希望这将支持患者和医疗保健专业人员在英国各地获得高标准的护理。