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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Patients with Cystic fibrosis | 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. | ||
| Patients with Primary ciliary dyskinesia | 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. | ||
| Healthy controls | 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. |
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| Measure | Description | Time Frame |
|---|---|---|
| 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. | First day |
| Measure | Description | Time Frame |
|---|---|---|
| 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. | First day |
| Pulmonary function (Forced expiratory volume in the first second (FEV1)) |
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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.
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Thirty-one patients with CF, 27 patients with PSD were included in the study. There are 30 healthy data.
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| Name | Affiliation | Role |
|---|---|---|
| Şeyma MUTLU KAYAARSLAN, MSc | Baskent University | Study Chair |
| Meral BOŞNAK GÜÇLÜ, Prof. Dr | Gazi University | Study Director |
| Betül YOLERİ, MSc | Gazi University | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Gazi University Faculty of Health Sciences Department of Physiotherapy ana Rehabilitation, Cardiopulmonary Rehabilitation Unit | Ankara | Çankaya | 06490 | Turkey (Türkiye) |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 11562511 | Background | Sharma R, Florea VG, Bolger AP, Doehner W, Florea ND, Coats AJ, Hodson ME, Anker SD, Henein MY. Wasting as an independent predictor of mortality in patients with cystic fibrosis. Thorax. 2001 Oct;56(10):746-50. doi: 10.1136/thorax.56.10.746. | |
| 27836958 | Background | Lucas JS, Barbato A, Collins SA, Goutaki M, Behan L, Caudri D, Dell S, Eber E, Escudier E, Hirst RA, Hogg C, Jorissen M, Latzin P, Legendre M, Leigh MW, Midulla F, Nielsen KG, Omran H, Papon JF, Pohunek P, Redfern B, Rigau D, Rindlisbacher B, Santamaria F, Shoemark A, Snijders D, Tonia T, Titieni A, Walker WT, Werner C, Bush A, Kuehni CE. European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesia. Eur Respir J. 2017 Jan 4;49(1):1601090. doi: 10.1183/13993003.01090-2016. Print 2017 Jan. |
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| ID | Term |
|---|---|
| D003550 | Cystic Fibrosis |
| D002925 | Ciliary Motility Disorders |
| ID | Term |
|---|---|
| D010182 | Pancreatic Diseases |
| D004066 | Digestive System Diseases |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
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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. |
| 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. | 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. | First day |
| Pulmonary function (Peak flow rate (PEF)) | 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. | 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. | 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. | 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. | 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. | 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. | 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. | 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. | 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. | 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. | 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. | 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. | 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. | 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. | 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. | 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. | 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. | 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. | Second day |
| 8630582 | Background | Gosselink R, Troosters T, Decramer M. Peripheral muscle weakness contributes to exercise limitation in COPD. Am J Respir Crit Care Med. 1996 Mar;153(3):976-80. doi: 10.1164/ajrccm.153.3.8630582. |
| 39785198 | Background | Mutlu S, Bosnak Guclu M, Sismanlar Eyuboglu T, Aslan AT. Upper Extremity Exercise Capacity and Muscle Oxygenation in Patients With Primary Ciliary Dyskinesia. Pediatr Pulmonol. 2025 Jan;60(1):e27470. doi: 10.1002/ppul.27470. |
| 30540111 | Background | Souza RP, Donadio MVF, Heinzmann-Filho JP, Baptista RR, Pinto LA, Epifanio M, Marostica PJC. THE USE OF ULTRASONOGRAPHY TO EVALUATE MUSCLE THICKNESS AND SUBCUTANEOUS FAT IN CHILDREN AND ADOLESCENTS WITH CYSTIC FIBROSIS. Rev Paul Pediatr. 2018 Oct-Dec;36(4):457-465. doi: 10.1590/1984-0462/;2018;36;4;00015. |
| 30049738 | Background | Halbeisen FS, Goutaki M, Spycher BD, Amirav I, Behan L, Boon M, Hogg C, Casaulta C, Crowley S, Haarman EG, Karadag B, Koerner-Rettberg C, Loebinger MR, Mazurek H, Morgan L, Nielsen KG, Omran H, Santamaria F, Schwerk N, Thouvenin G, Yiallouros P, Lucas JS, Latzin P, Kuehni CE. Lung function in patients with primary ciliary dyskinesia: an iPCD Cohort study. Eur Respir J. 2018 Aug 23;52(2):1801040. doi: 10.1183/13993003.01040-2018. Print 2018 Aug. |
| 27259460 | Background | Harun SN, Wainwright C, Klein K, Hennig S. A systematic review of studies examining the rate of lung function decline in patients with cystic fibrosis. Paediatr Respir Rev. 2016 Sep;20:55-66. doi: 10.1016/j.prrv.2016.03.002. Epub 2016 Mar 14. |
| 27189798 | Background | Ratjen F, Bell SC, Rowe SM, Goss CH, Quittner AL, Bush A. Cystic fibrosis. Nat Rev Dis Primers. 2015 May 14;1:15010. doi: 10.1038/nrdp.2015.10. |
| D030342 |
| Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D007232 | Infant, Newborn, Diseases |
| D010038 | Otorhinolaryngologic Diseases |
| D000072661 | Ciliopathies |
| D000015 | Abnormalities, Multiple |
| D000013 | Congenital Abnormalities |