与杜氏肌肉发育不良症呼吸系统健康和功能相关的因素:系统性审查和证据分级
E Landfeldt1, A Aleman2,3, S Abner4
1IQVIA, Stockholm, Sweden.
Journal of neuromuscular diseases
|November 19, 2023
概括
呼吸系统问题显著影响杜申肌力发育不良症 (DMD) 患者. 本综述确定了10个关键因素,包括遗传学和治疗方法,影响DMD的呼吸系统健康,指导未来的研究和护理.
科学领域:
- 神经学 神经学
- 肺部病理学 肺部病理学
- 遗传学 遗传学 是一个
背景情况:
- 呼吸道并发症是杜氏肌肉发育不良 (DMD) 发病率和死亡的主要原因.
- 尽管医学取得了进步,但了解影响DMD呼吸系统健康的因素对于患者的治疗结果至关重要.
研究的目的:
- 系统地审查和评分与杜申尼肌肉衰竭中呼吸系统健康和功能相关的因素的证据.
- 在DMD中合成呼吸道疾病进展的预后指标和预测指标.
主要方法:
- 在2000-2022年期间,对MEDLINE,Embase和Cochrane图书馆进行了全面的文献搜索.
- 包括29篇关于DMD呼吸系统健康预后指标的文章.
- 使用GRADE框架进行证据质量评估.
主要成果:
- 确定了影响DMD呼吸系统健康的10个因素.
- 关键因素包括葡萄糖皮质激素暴露,DMD突变,基因修饰剂,药理干预 (例如,阿塔卢伦,埃特普利尔森),体重指数,体重和功能能力.
- 证据质量对于已识别的因素从非常低到很高不等.
结论:
- 十个因素,包括遗传学,疗法和临床特征,与DMD的呼吸系统健康有关.
- 需要进一步研究基因型-表型相关性和新的DMD疗法的现实影响.
- 这些发现旨在为临床实践提供信息,并指导对DMD患者群体的未来研究.
相关概念视频
Assessment of Airway, Skin Color, and Use of Accessory Muscles
1.0K
A thorough assessment of respiratory health is paramount in clinical settings to identify and manage respiratory distress and ensure adequate oxygenation. This article elaborates on the critical aspects of respiratory evaluation, including airway assessment, skin color examination, and the observation of accessory muscle use, which are integral to effectively diagnosing and managing patients with respiratory conditions.
Introduction
The initial evaluation of a patient's respiratory system...
Introduction
The initial evaluation of a patient's respiratory system...
1.0K
Factors Affecting Pulmonary Ventilation
1.4K
Besides the pressure difference between the external environment and the lungs, the airflow rate and ease of pulmonary ventilation are also influenced by three other factors: surface tension of the fluid in the alveoli, compliance of the lungs, and airway resistance.
Alveolar Surface Tension
The alveolar fluid lines the luminal surface of the alveoli and exerts a force called surface tension. This force is caused by the polar water molecules in the liquid being more strongly attracted to each...
Alveolar Surface Tension
The alveolar fluid lines the luminal surface of the alveoli and exerts a force called surface tension. This force is caused by the polar water molecules in the liquid being more strongly attracted to each...
1.4K
Physical Assessment of the Respiratory Tract II: Inspection
306
Physical assessment of the respiratory tract through inspection is a crucial step in understanding the patient's respiratory health. It provides insights into the functioning of the respiratory system, the musculoskeletal structure, and even the patient's nutritional status. This comprehensive approach involves observing several vital aspects: chest configuration, breathing patterns, respiratory rates, skin color, and use of accessory muscles.
Chest Configuration
The chest configuration...
Chest Configuration
The chest configuration...
306
Respiratory System Abnormal Finding I: Inspection and Percussion
276
Respiratory system abnormalities are a significant concern in healthcare due to their potential to indicate underlying severe conditions like Chronic Obstructive Pulmonary Disease (COPD), asthma, and pneumonia. These abnormalities can often be detected through physical examination methods like inspection and percussion.
Inspection Findings
During an inspection, several findings may suggest the presence of respiratory distress or disease. Pursed-lip breathing, where exhalation is slowed by...
Inspection Findings
During an inspection, several findings may suggest the presence of respiratory distress or disease. Pursed-lip breathing, where exhalation is slowed by...
276
Assessment of Respiration
1.1K
The respiratory system's basic structures and primary functions lay the foundation for nurses' comprehensive respiratory assessments. This assessment includes subjective and objective data to gauge the patient's respiratory health.
Subjective Assessment: Nurses interview the patient to gather information directly during the subjective assessment. It includes questions about the individual's medical history, medications, and symptoms, focusing on past respiratory conditions like...
Subjective Assessment: Nurses interview the patient to gather information directly during the subjective assessment. It includes questions about the individual's medical history, medications, and symptoms, focusing on past respiratory conditions like...
1.1K
Chronic Obstructive Pulmonary Disease-II: Pathophysiology
2.8K
Chronic Obstructive Pulmonary Disease (COPD) pathophysiology is intricate and multifaceted, involving a complex interplay of physiological processes. Understanding these mechanisms is crucial for effectively managing and treating COPD. Here is an in-depth look at the critical elements in the pathophysiology of COPD:
Chronic Inflammation
Chronic Inflammation
2.8K


