Department of Cardiopulmonary Physiotherapy and Rehabilitation, Division of Physiotherapy and Rehabilitation, Faculty of Health Sciences, Bezmialem Vakif University, Istanbul, Turkey.
Division of Physiotherapy and Rehabilitation, Faculty of Health Sciences, Izmir Bakircay University, Izmir, Turkey.
Pediatr Pulmonol. 2020 Nov;55(11):3067-3073. doi: 10.1002/ppul.25052. Epub 2020 Sep 11.
Primary ciliary dyskinesia (PCD) is generally likened to cystic fibrosis (CF) due to similarities in impaired mucociliary clearance and some other symptoms. The aim of our study was to investigate pulmonary and extrapulmonary characteristics of children with CF and PCD since no studies have addressed respiratory muscle strength in children with PCD and to compare the results to those obtained from healthy age-matched controls.
Pulmonary and extrapulmonary characteristics were assessed by 6-min walk test, spirometry, maximum inspiratory and expiratory pressure measurements, and knee extensor strength test in the children with CF, PCD, and healthy controls.
Children with PCD and CF had similar PFT results, except forced expiratory flow between 25% and 75% of vital capacity (FEF ) which was lower in PCD (p = .04). Maximum inspiratory pressure (MIP) value was lower in the children with CF compared with the healthy controls (p = .016), MEP value of the children with PCD was worse than those with CF and healthy controls (p = .013 and p = .013), respectively. 6-min walk test (6MWT) distance of the children with CF was lower than their healthy counterparts (p = .003). Knee extensor muscle strength differed among the children with PCD, CF, and healthy control groups, but post hoc test failed to show statistical significance (p = .010).
Children with CF and PCD had some impairments in pulmonary functions, respiratory muscle strength, functional capacity, and peripheral muscle strength compared with healthy children. However, the unique characteristics of each disease should be considered during physiotherapy assessment and treatment. The clinicians may especially focus on the respiratory and peripheral muscle strength of the children with PCD.
原发性纤毛运动障碍（PCD）通常由于黏液纤毛清除功能受损和其他一些症状相似而类似于囊性纤维化（CF）。我们的研究目的是研究 CF 和 PCD 患儿的肺外和肺特征，因为尚无研究涉及 PCD 患儿的呼吸肌力量，并将结果与健康年龄匹配的对照组进行比较。
通过 6 分钟步行测试、肺活量测定、最大吸气和呼气压力测量以及膝伸肌力量测试评估 CF、PCD 和健康对照组儿童的肺外和肺特征。
PCD 和 CF 患儿的 PFT 结果相似，除了用力呼气流量 25%至 75%肺活量（FEF）外，PCD 患儿的 FEF 较低（p=0.04）。与健康对照组相比，CF 患儿的最大吸气压力（MIP）值较低（p=0.016），PCD 患儿的 MEP 值较 CF 患儿和健康对照组差（p=0.013 和 p=0.013）。CF 患儿的 6 分钟步行测试（6MWT）距离低于其健康同龄人（p=0.003）。PCD、CF 和健康对照组儿童的膝伸肌肌肉力量存在差异，但事后检验未显示出统计学意义（p=0.010）。
与健康儿童相比，CF 和 PCD 患儿的肺功能、呼吸肌力量、功能能力和外周肌肉力量存在一些损害。然而，在物理治疗评估和治疗过程中应考虑每种疾病的独特特征。临床医生可能特别关注 PCD 患儿的呼吸和外周肌肉力量。
