The number of studies evaluating respiratory functions and upper extremity functional exercise capacity in patients with CF and PCD is limited in the literature. There are no studies comparing upper extremity functional exercise capacity and upper extremity muscle oxygenation in patients with CF and PCD. The aim of our study is to compare respiratory function, upper extremity functional exercise capacity, peripheral muscle strength, muscle oxygenation, and balance in CF, PCD, and healthy children.
Cystic fibrosis (CF) is an autosomal recessive, monogenic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. In individuals with CF, infections, inflammation, mucus plugging, nutritional deficiencies, pulmonary exacerbations, and respiratory tract diseases contribute to a decline in pulmonary function. Increased airway resistance, air trapping, and reduced airflow rates lead to a decrease in lung capacities. Primary ciliary dyskinesia (PCD) is a genetically heterogeneous disorder caused by the absence or dysfunction of motile cilia. In PCD, pulmonary function is affected from early in life and remains below reference values across all age groups and both sexes. Decreased muscle strength has been demonstrated in both CF and PCD patients. In chronic obstructive pulmonary disease (COPD), pulmonary function and limb muscle strength are important determinants of exercise capacity. There is a limited number of studies in the literature assessing pulmonary function and upper extremity functional exercise capacity in patients with CF and PCD. Furthermore, to our knowledge, no studies have compared upper extremity functional exercise capacity and upper extremity muscle oxygenation between patients with CF and PCD. The aim of this study is to compare pulmonary function, upper extremity functional exercise capacity, peripheral muscle strength, muscle oxygenation, and balance among children with CF, children with PCD, and healthy controls. The study was planned as cross-sectional and retrospective. The patient group will include individuals diagnosed with CF and PCD, aged between 6 and 18 years, receiving standard medical treatment. The healthy group will include individuals aged between 6 and 18 years without any known chronic disease. Individuals' respiratory functions, upper extremity functional exercise capacity, peripheral muscle strength, muscle oxygenation, and balance will be evaluated. Upper extremity functional exercise capacity was evaluated using the 6-minute pegboard and ring test, pulmonary function using spirometry, peripheral muscle strength using a hand-held dynamometer, muscle oxygenation using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD), static balance using the "Biosway Portable Balance System", and dynamic balance assessment using the Y balance test. The assessments will be completed in two days.
Gazi University Faculty of Health Sciences Department of Physiotherapy ana Rehabilitation, Cardiopulmonary Rehabilitation Unit
Ankara, Çankaya, Turkey (Türkiye)
Upper extremity functional exercise capacity
Upper extremity functional exercise capacity was assessed with a 6-minute pegboard and ring test (6-PBRT). Subjects were instructed to move as many rings as possible for six minutes. Before and after each test and during the first minute of recovery, HR, SpO2, blood pressure, respiratory frequency, perceived dyspnea, and fatigue were recorded. Total ring counts were recorded as the outcome measure.
Time frame: First day
Pulmonary function (Forced vital capacity (FVC))
Pulmonary function was assesed with the spirometry. Dynamic lung volume measurements were made according to ATS and ERS criteria. With the device, forced vital capacity (FVC) was assessed.
Time frame: First day
Pulmonary function (Forced expiratory volume in the first second (FEV1))
Pulmonary function was assessed with the spirometry. Dynamic lung volume measurements were made according to ATS and ERS criteria. With the device, forced expiratory volume in the first second (FEV1) was assessed.
Time frame: First day
Pulmonary function (FEV1 / FVC)
Pulmonary function was assessed with the spirometry. Dynamic lung volume measurements were made according to ATS and ERS criteria. With the device, FEV1 / FVC was assessed.
Time frame: First day
Pulmonary function (Flow rate 25-75% of forced expiratory volume (FEF 25-75%))
Pulmonary function was assessed with the spirometry. Dynamic lung volume measurements were made according to ATS and ERS criteria. With the device, flow rate 25-75% of forced expiratory volume (FEF 25-75%) was assessed.
Time frame: First day
Pulmonary function (Peak flow rate (PEF))
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Study Type
OBSERVATIONAL
Enrollment
88
Pulmonary function was assessed with the spirometry. Dynamic lung volume measurements were made according to ATS and ERS criteria. With the device, peak flow rate (PEF) was assessed.
Time frame: First day
Peripheral muscle strength
Quadriceps and deltoid muscle strength was assessed using a hand dynamometer (JTECH Power Track Commander, Baltimore, USA). The assessments for both the right and left sides were repeated three times.
Time frame: Second day
Muscle oxygenation (SmO2rest)
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6PBRT, the device was placed on the deltoid muscle of the dominant arm, and measurements were recorded.
Time frame: First day
Muscle oxygenation (SmO2min)
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6PBRT, the device was placed on the deltoid muscle of the dominant arm, and measurements were recorded.
Time frame: First day
Muscle oxygenation (SmO2max)
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6PBRT, the device was placed on the deltoid muscle of the dominant arm, and measurements were recorded.
Time frame: First day
Muscle oxygenation (Δ SmO2)
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6PBRT, the device was placed on the deltoid muscle of the dominant arm, and measurements were recorded.
Time frame: First day
Muscle oxygenation (SmO2averaged-min)
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6PBRT, the device was placed on the deltoid muscle of the dominant arm, and measurements were recorded.
Time frame: First day
Muscle oxygenation (SmO2averaged -max)
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6PBRT, the device was placed on the deltoid muscle of the dominant arm, and measurements were recorded.
Time frame: First day
Muscle oxygenation (Δ SmO2averaged)
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6PBRT, the device was placed on the deltoid muscle of the dominant arm, and measurements were recorded.
Time frame: First day
Muscle oxygenation (SmO2recovery)
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6PBRT, the device was placed on the deltoid muscle of the dominant arm, and measurements were recorded.
Time frame: First day
Muscle oxygenation (SmO2recovery-averaged)
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6PBRT, the device was placed on the deltoid muscle of the dominant arm, and measurements were recorded.
Time frame: First day
Muscle oxygenation (THbrest)
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6PBRT, the device was placed on the deltoid muscle of the dominant arm, and measurements were recorded.
Time frame: First day
Muscle oxygenation (THbmin)
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6PBRT, the device was placed on the deltoid muscle of the dominant arm, and measurements were recorded.
Time frame: First day
Muscle oxygenation (Thbmax)
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6PBRT, the device was placed on the deltoid muscle of the dominant arm, and measurements were recorded.
Time frame: First day
Muscle oxygenation (Δ THb)
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6PBRT, the device was placed on the deltoid muscle of the dominant arm, and measurements were recorded.
Time frame: First day
Muscle oxygenation (THbrecovery)
Muscle oxygenation was assessed using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD). During the 6PBRT, the device was placed on the deltoid muscle of the dominant arm, and measurements were recorded.
Time frame: First day
Static Balance
The static balance evaluated individuals using the "Biosway Portable Balance System" (Biodex Medical Systems Incorporated, Shirley, New York). In the evaluations, the test was repeated three times with a test duration of 20 seconds, and a 10-second inter-evaluation was permitted. The test was repeated under the conditions of eyes open, hard ground, eyes closed, hard ground, eyes open, soft ground, and eyes closed, soft ground.
Time frame: Second day
Dynamic balance
The dynamic balance assessment was evaluated using the Y balance test. The evaluations were repeated three times for both lower extremities in the anterior, posteromedial, and posterolateral directions. While the individuals maintained a single-leg stance on the right side, the left foot was extended as far as possible along the measuring tape, with the toe positioned in the anterior, posteromedial, and posterolateral directions. The same procedure was repeated on the other side.
Time frame: Second day