| 标题： | 杜氏肌营养不良儿童呼吸康复策略专家共识 |
| title： | Expert Consensus on Respiratory Rehabilitation Strategies for Children with Duchenne Muscular Dystrophy |
| 版本： | 原创版 |
| version： | Original |
| 分类： | 专家共识 |
| classification： | Experts consensus |
| 领域： | 治疗 |
| field： | Treatment |
| 国家和地区： | 中国 |
| Country and region： | China |
| 指南使用者： | 1.医疗机构内从事儿科、康复医学、呼吸治疗等相关科室的医护人员。 2.患儿家长及监护人，帮助他们理解康复的重要性并积极参与。 3.公共卫生机构、医疗保险机构及政策制定部门，用于指导资源配置和政策制 |
| Guide users： | 1.Medical staff in the departments of pediatrics, rehabilitation medicine, respiratory therapy, and other related departments in medical institutions. 2.Parents and guardians of children should help them understand the importance of rehabilitation and actively participate in it. 3.Public health institutions, medical insurance institutions and policy-making departments, used to guide resource allocation and policy formulation. |
| 证据分级方法： | 采用系统评级证据方法（如GRADE系统）来评估和分析现有研究证据，确保推荐建议的科学性和可靠性。具体步骤包括： 1.文献检索：全面检索国内外相关数据库，收集关于DMD患儿呼吸康复的最新研究成果。 2.证据评价：根据研究设计、样本量、结果一致性等因素，对收集到的证据进行质量评价。 3.推荐形成：基于系统评价的结果，结合专家共识，形成具体的推荐建议。 4.外部评审：邀请相关领域的专家对指南草案进行评审，确保推荐建议的准确性和适用性。 |
| Evidence grading method： | This guide will use systematic rating evidence methods such as the GRADE system to evaluate and analyze existing research evidence, ensuring the scientific validity and reliability of the recommendations. Specific steps include: Literature search: comprehensively search relevant databases at home and abroad to collect the latest research results on respiratory rehabilitation for children with DMD. Evidence evaluation: evaluate the quality of collected evidence based on factors such as study design, sample size, and consistency of results. Recommendation formation: Based on the results of systematic evaluation and expert consensus, specific recommendations are formed. External review: experts in relevant fields are invited to review the draft guidelines to ensure the accuracy and applicability of the recommendations. |
| 制定单位： | 四川大学华西第二医院 |
| Formulating unit： | West China Second Hospital of Sichuan University |
| 注册时间： | 2024-09-12 |
| Registration time： | |
| 注册编号： | PREPARE-2024CN495 |
| Registration number： | |
| 指南制订的目的： | DMD是一种X染色体连锁隐性遗传疾病，由编码抗肌萎缩蛋白的DMD基因致病性变异所导致的一组主要累及骨骼肌和（或）心肌的X-连锁隐性遗传性肌病。随着病程的进展，DMD患者的呼吸肌肌力减弱，胸廓顺应性降低，进而引发一系列呼吸并发症，如咳嗽无力、肺通气不足、膈肌纤维化、反复呼吸道感染及睡眠相关呼吸问题等。呼吸并发症是DMD患者的主要死因之一，严重影响患者的生活质量和预期寿命。本指南旨在为杜氏肌营养不良（DMD）患儿，提供一套科学、全面、系统的呼吸康复策略，通过整合国内外最新研究成果和临床实践经验，规范DMD孩子呼吸康复评估及治疗流程，提高DMD孩子呼吸康复管理水平，减少呼吸并发症，延长DMD患儿生存时间，改善生活质量。 |
| Purpose of the guideline： | DMD is a X-linked recessive genetic disease caused by pathogenic mutations in the DMD gene, which encodes dystrophin, leading to a group of X-linked recessive genetic myopathies primarily affecting skeletal and/or cardiac muscles. As the disease progresses, the respiratory muscle strength of DMD patients weakens and the thoracic compliance decreases, which leads to a series of respiratory complications, such as weak coughing, insufficient pulmonary ventilation, diaphragmatic fibrosis, recurrent respiratory tract infections, and sleep-related respiratory problems. Respiratory complications are one of the main causes of death in patients with DMD, which seriously affects the quality of life and life expectancy of patients. This guide aims to provide a scientific, comprehensive, and systematic respiratory rehabilitation strategy for children with Duchenne muscular dystrophy (DMD). By integrating the latest research results and clinical experience from home and abroad, it standardizes the respiratory rehabilitation evaluation and treatment process for DMD children, improves the management level of respiratory rehabilitation for DMD children, reduces respiratory complications, prolongs the survival time of DMD children, and improves their quality of life. |
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