Cystic Fibrosis (CF), Primary Ciliary Dyskinesia (PCD)
Conditions
Keywords
cystic fibrosis, primary ciliary dykinesia, balance, muscle oxygenation, exercise tolerance, upper extremity
Brief summary
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.
Detailed description
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.
Interventions
None listed
Sponsors
Gazi University
Study design
Observational model
OTHER
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
6 Years to 18 Years
Healthy volunteers
Yes
Inclusion criteria
Cystic fibrosis patients • Patients between the ages of 6-18 years who are diagnosed with cystic fibrosis according to the American Cystic Fibrosis Association consensus report and whose clinical condition is stable will be included in the study. Primary ciliary dyskinesia patients • Patients between the ages of 6-18 years who are diagnosed with primary ciliary dyskinesia according to the American Thoracic Society (ATS) and European Respiratory Society (ERS) guidelines and whose clinical condition was stable will be included in the study. Healthy controls • Healthy individuals of both sexes between the ages of 6-18 who voluntarily agreed to participate in the study will be included.
Exclusion criteria
Patients •Patients who are uncooperative, have orthopedic or neurological disorders that will affect functional capacity, and have pneumonia or any acute infection during the evaluation will be excluded from the study. Healthy controls; •Those with a known chronic disease, uncooperative and orthopedic or neurological disorders that will affect functional capacity will not be included.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Upper extremity functional exercise capacity | First day | 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. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Pulmonary function (Forced expiratory volume in the first second (FEV1)) | First day | 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. |
| Pulmonary function (FEV1 / FVC) | First day | 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. |
| Pulmonary function (Flow rate 25-75% of forced expiratory volume (FEF 25-75%)) | First day | 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. |
| Pulmonary function (Peak flow rate (PEF)) | First day | 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. |
| Peripheral muscle strength | Second day | 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. |
| Muscle oxygenation (SmO2rest) | First day | 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. |
| Muscle oxygenation (SmO2min) | First day | 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. |
| Muscle oxygenation (SmO2max) | First day | 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. |
| Muscle oxygenation (Δ SmO2) | First day | 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. |
| Muscle oxygenation (SmO2averaged-min) | First day | 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. |
| Pulmonary function (Forced vital capacity (FVC)) | First day | 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. |
| Muscle oxygenation (Δ SmO2averaged) | First day | 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. |
| Muscle oxygenation (SmO2recovery) | First day | 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. |
| Muscle oxygenation (SmO2recovery-averaged) | First day | 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. |
| Muscle oxygenation (THbrest) | First day | 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. |
| Muscle oxygenation (THbmin) | First day | 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. |
| Muscle oxygenation (Thbmax) | First day | 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. |
| Muscle oxygenation (Δ THb) | First day | 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. |
| Muscle oxygenation (THbrecovery) | First day | 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. |
| Static Balance | Second day | 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. |
| Dynamic balance | Second day | 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. |
| Muscle oxygenation (SmO2averaged -max) | First day | 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. |
Countries
Turkey (Türkiye)
Outcome results
None listed