Expert Consensus on Respiratory Rehabilitation Strategies for Children with Duchenne Muscular Dystrophy

Title: Expert Consensus on Respiratory Rehabilitation Strategies for Children with Duchenne Muscular Dystrophy
Edition: Original
Classification: Experts consensus
Field: Treatment
Countries and regions: China
Guidelines 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.
Evidence classification 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.
Development unit: West China Second Hospital of Sichuan University
Registration time: 2024-09-12
Registration number: PREPARE-2024CN495
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.