Sonbahar-Ulu Hazal, Cakmak Aslihan, Inal-Ince Deniz, Vardar-Yagli Naciye, Yatar Ilker, Calik-Kutukcu Ebru, Saglam Melda, Tekerlek Haluk, Bozdemir-Ozel Cemile, Arikan Hulya, Emiralioglu Nagehan, Ozcelik Ugur
Faculty of Physical Therapy and Rehabilitation, Hacettepe University, Ankara, Turkey.
Department of Physiotherapy and Rehabilitation, Faculty of Health Sciences, Eastern Mediterranean University, Famagusta, Cyprus.
Pediatr Int. 2022 Jan;64(1):e14979. doi: 10.1111/ped.14979.
Primary ciliary dyskinesia (PCD) is a rare, hereditary, autosomal recessive disorder characterized by recurrent upper and lower respiratory tract infections. This study aimed to compare pulmonary function, respiratory muscle strength, exercise capacity, physical fitness, and activities of daily living (ADLs) retrospectively between patients with PCD and healthy controls.
Data from 20 patients with PCD and 20 matched, healthy counterparts recorded between July 2015 and January 2017 were analyzed. The data evaluated and recorded included pulmonary function using a portable spirometer, respiratory muscle strength (using a mouth pressure device, exercise capacity using the incremental shuttle walk test,), physical fitness (using the Munich Fitness Test), and ADLs (using the Glittre ADL test).
Pulmonary function, respiratory muscle strength, incremental shuttle walk test scores (all parameters), and total Munich Fitness Test scores were lower in the PCD group relative to controls (P < 0.05). The duration to complete the Glittre ADL test was higher in PCD patients than in healthy subjects (P < 0.05).
Pulmonary function, respiratory muscle strength, exercise capacity, physical fitness, and ADL were affected by PCD. Thus, pulmonary rehabilitation regimens should be tailored according to these impacts.
原发性纤毛运动障碍（PCD）是一种罕见的遗传性常染色体隐性疾病，其特征为反复的上、下呼吸道感染。本研究旨在回顾性比较PCD患者与健康对照者之间的肺功能、呼吸肌力量、运动能力、身体素质和日常生活活动（ADL）。
分析2015年7月至2017年1月期间记录的20例PCD患者和20例匹配的健康对照者的数据。评估和记录的数据包括使用便携式肺活量计测量的肺功能、呼吸肌力量（使用口腔压力装置）、使用递增往返步行试验测量的运动能力、身体素质（使用慕尼黑体能测试）以及ADL（使用Glittre ADL测试）。
与对照组相比，PCD组的肺功能、呼吸肌力量、递增往返步行试验得分（所有参数）以及慕尼黑体能测试总得分均较低（P < 0.05）。PCD患者完成Glittre ADL测试的时间比健康受试者更长（P < 0.05）。
PCD会影响肺功能、呼吸肌力量、运动能力、身体素质和ADL。因此，应根据这些影响来制定肺康复方案。
