Firat Merve, Bosnak-Guclu Meral, Sismanlar-Eyuboglu Tugba, Tana-Aslan Ayse
Hacettepe University, Faculty of Physical Therapy and Rehabilitation, Ankara, Turkey.
Gazi University, Faculty of Health Sciences, Department of Physical Therapy and Rehabilitation, Ankara, Turkey.
Respir Med. 2022 Jan;191:106719. doi: 10.1016/j.rmed.2021.106719. Epub 2021 Dec 17.
The pathophysiological mechanisms of primary ciliary dyskinesia (PCD) may affect many functions, including respiratory, physical, and health status. This study aimed to compare respiratory muscle strength, inspiratory muscle endurance, muscle strength, exercise capacity, physical activity levels, and quality of life in PCD patients and controls.
Twenty-seven patients and 28 controls were included. Respiratory muscle strength (maximal inspiratory (MIP) and maximal expiratory (MEP) pressures), inspiratory muscle endurance (incremental threshold loading test), muscle strength (quadriceps femoris, shoulder abductor, elbow flexor, handgrip), exercise capacity (6-min walk test (6MWT)), physical activity and quality of life (QOL-PCD) were evaluated.
MIP, inspiratory muscle endurance, quadriceps femoris, and handgrip muscle strength, 6MWT distance, total energy expenditure; childrens' (6-12 years) and their parents' physical function, upper, lower respiratory, and hearing symptoms and treatment burden QOL-PCD subscales scores were significantly lower in patients compared with controls (p < 0.05). The 66.7% of patients did not meet the optimal number of steps. MEP, shoulder abductor, and elbow flexor muscle strength, active energy expenditure, physical activity duration, average metabolic equivalents, number of steps, lying time, and sleep duration; childrens' and their parents' other subscales and adolescents' all QOL-PCD subscales scores were similar between groups (p > 0.05).
Inspiratory muscle strength and endurance, lower extremity and total muscle strength, exercise capacity, total energy expenditure, and childrens' quality of life are impaired compared to healthy controls. Decreased physical activity level is prevalent in these patients. Effects of pulmonary rehabilitation on these impaired outcomes for PCD patients should be investigated.
Clinicaltrials.gov: NCT03370029; December 12, 2017.
原发性纤毛运动障碍（PCD）的病理生理机制可能影响多种功能，包括呼吸、身体和健康状况。本研究旨在比较PCD患者与对照组的呼吸肌力量、吸气肌耐力、肌肉力量、运动能力、身体活动水平和生活质量。
纳入27例患者和28例对照。评估呼吸肌力量（最大吸气（MIP）和最大呼气（MEP）压力）、吸气肌耐力（递增阈值负荷试验）、肌肉力量（股四头肌、肩外展肌、肘屈肌、握力）、运动能力（6分钟步行试验（6MWT））、身体活动和生活质量（QOL-PCD）。
与对照组相比，患者的MIP、吸气肌耐力、股四头肌和握力、6MWT距离、总能量消耗；儿童（6 - 12岁）及其父母的身体功能、上、下呼吸道和听力症状以及治疗负担QOL-PCD子量表得分显著降低（p < 0.05）。66.7%的患者未达到最佳步数。MEP、肩外展肌和肘屈肌力量、活动能量消耗、身体活动持续时间、平均代谢当量、步数、躺卧时间和睡眠时间；儿童及其父母的其他子量表以及青少年的所有QOL-PCD子量表得分在两组之间相似（p > 0.05）。
与健康对照组相比，PCD患者的吸气肌力量和耐力、下肢及全身肌肉力量、运动能力、总能量消耗以及儿童的生活质量受损。这些患者普遍存在身体活动水平下降的情况。应研究肺康复对PCD患者这些受损结局的影响。
Clinicaltrials.gov：NCT03370029；2017年12月12日。
