Clinical trial NCT04895150: Upper Extremity Exercise Capacity, Muscle Oxygenation, Balance in Patients With Primary Ciliary Dyskinesia
Official title: Investigation of Upper Extremity Exercise Capacity, Muscle Oxygenation, Balance and Physical Activity Levels in Patients With Primary Ciliary Dyskinesia
Study type: OBSERVATIONAL
Overall status: COMPLETED
Conditions: Primary Ciliary Dyskinesia
Keywords: Primary Ciliary Dyskinesia
Upper Extremity Exercise Capacity
Muscle Oxygenation
Balance
Physical Activity
Lead sponsor: Gazi University
Sponsor class: OTHER
First posted: 2021-05-20
Enrollment: 54
Sex: ALL
Minimum age (years): 6
Maximum age (years): 18
Standard ages: CHILD
ADULT
Accepts healthy volunteers: yes
Observational model: CASE_CONTROL
Time perspective: CROSS_SECTIONAL
Sampling method: NON_PROBABILITY_SAMPLE
Study population: At least 18 patients with primary ciliary dyskinesia will be included in patients group and 18 healthy individuals will be included in control group.

Brief summary: The main aim of the study is to evaluate upper extremity exercise capacity and muscle oxygenation in patients with primary ciliary dyskinesia. The secondary aim of the study is to evaluate respiratory function, respiratory muscle strength and endurance, peripheral muscle strength, balance, physical activity level and quality of life in patients with primary ciliary dyskinesia and compare all parameters with healthy controls.

Detailed description: Primary ciliary dyskinesia (PCD) is a genetic, heterogeneous disease caused by cilia absence or dysfunction. Impaired function of motile cilia leads to impaired mucociliary clearance, recurrent chest infections, and progressive destruction of the lung structure. Clinical features include neonatal respiratory distress syndrome, chronic daily productive cough, chronic nasal congestion, and chronic or recurrent otitis media.

Pulmonary functions, exercise capacity, respiratory muscle strength and endurance, peripheral muscle strength, physical activity level and quality of life were affected in patients. The number of studies on these subjects is limited.

Pulmonary function and extremity muscle strength are determinants of exercise capacity in chronic obstructive pulmonary disease. Decreased strength and endurance in the limb muscles is associated with exercise intolerance. Patients have decreased upper extremity muscle strength. There is no study in the literature evaluating upper extremity exercise capacity in patients with PCD. Measuring the oxygen saturation of cardiorespiratory and peripheral muscles is important for understanding the dynamics of oxygen delivery and consumption during exercise. There are no studies in the literature evaluating muscle oxygenation in this patient population.

The main aim of the study is to evaluate upper extremity exercise capacity and muscle oxygenation in patients with primary ciliary dyskinesia. The secondary aim of the study is to evaluate respiratory function, respiratory muscle strength and endurance, peripheral muscle strength, balance, physical activity level and quality of life in patients with primary ciliary dyskinesia and compare them with healthy controls.

The study was planned cross-sectional. Patients with primary ciliary dyskinesia referred to the Cardiopulmonary Rehabilitation Unit of Gazi University Physiotherapy and Rehabilitation Department by the physicians of the Department of Pediatric Pulmonary Diseases of Gazi University Faculty of Medicine will be included in the study. At least 18 patients with primary ciliary dyskinesia and at least 18 healthy controls of similar age and sex will be evaluated in the study. The assessments will be completed in two days.

Upper extremity exercise capacity (6 minutes Pegboard and Ring Test), muscle oxygenation ("Moxy" monitor), functional exercise capacity(6 minutes walk test), respiratory functions (spirometer), respiratory muscle strength (mouth pressure measurement), respiratory muscle endurance (incremental threshold loading test), peripheral muscle strength (dynamometer), balance("Biodex Balance System®" and Y balance test), physical activity level (multi-sensor activity monitor) and quality of life (Quality of Life scale for Primary Ciliary Dyskinesia (QOL-PCD scale version 4.3) will be evaluated.

Eligibility criteria: Inclusion Criteria:

Patients;

* 6-18 years old
* Diagnosed with primary ciliary dyskinesia
* Stability of clinical condition

Healthy controls;

* 6-18 years old

Exclusion Criteria:

Patients;

* Diagnosed neurological, cognitive problem that can affect evaluations,
* Acute pneumonia or any infection
* History of exacerbation in the last 1 month
* More than 10% change in FEV1 in the last 6 months
* History of coronavirus disease (COVID-19)
* History of smoking
* Diagnosed vision, hearing, vestibular, or neurological problems that can affect balance
* Diagnosed orthopedic problems affecting mobility or a history of musculoskeletal surgery

Healthy controls;

* Diagnosed neurological, cognitive problem that can affect evaluations
* Diagnosed vision, hearing, vestibular, or neurological problems that can affect balance
* History of coronavirus disease (COVID-19)
* History of smoking
* Trouble understanding and following the exercise test instruction

Primary outcomes:
measure: Upper extremity exercise capacity
timeFrame: First Day
measure: Muscle Oxygenation
timeFrame: First Day
Secondary outcomes:
measure: Functional exercise capacity
timeFrame: First Day
measure: Balance
timeFrame: Second day
measure: Physical activity (Total energy expenditure)
timeFrame: Second day
measure: Physical activity (Active energy expenditure (joule / day)
timeFrame: Second day
measure: Physical activity (Physical activity time (min / day)
timeFrame: Second Day
measure: Physical activity (Average metabolic equivalent (MET / day)
timeFrame: Second Day
measure: Physical activity (Number of steps (steps / day)
timeFrame: Second Day
measure: Physical activity (Time spent lying down (min / day) days)
timeFrame: Second Day
measure: Physical activity (Sleep time (min / day)
timeFrame: Second Day
measure: Respiratory muscle strength
timeFrame: First day
measure: Respiratory muscle endurance
timeFrame: Second Day
measure: Pulmonary function (Forced vital capacity (FVC))
timeFrame: First Day
measure: Pulmonary function (Forced expiratory volume in the first second (FEV1))
timeFrame: First Day
measure: Pulmonary function (FEV1 / FVC)
timeFrame: First Day
measure: Pulmonary function (Flow rate 25-75% of forced expiratory volume (FEF 25-75%))
timeFrame: First Day
measure: Pulmonary function (Peak flow rate (PEF))
timeFrame: First Day
measure: Life quality
timeFrame: Second day
Other outcomes:
Locations:
city: Ankara
country: Turkey
facility: Gazi University, Faculty of Health Science
city: Ankara
country: Turkey
facility: Gazi University
Study officials:
affiliation: Gazi University
name: Meral Boşnak Güçlü, Prof.Dr.
role: STUDY_DIRECTOR
affiliation: Gazi University
name: Şeyma Mutlu, Pt.
role: STUDY_CHAIR
affiliation: Gazi University
name: Tuğba ŞİŞMANLAR EYÜBOĞLU, Asc.Prof.Dr.
role: PRINCIPAL_INVESTIGATOR
affiliation: Gazi University
name: Ayşe Tana ARSLAN, Prof.Dr.
role: PRINCIPAL_INVESTIGATOR
References:
citation: Valerio G, Giallauria F, Montella S, Vaino N, Vigorito C, Mirra V, Santamaria F. Cardiopulmonary assessment in primary ciliary dyskinesia. Eur J Clin Invest. 2012 Jun;42(6):617-22. doi: 10.1111/j.1365-2362.2011.02626.x. Epub 2011 Nov 29.
citation: Ring AM, Buchvald FF, Holgersen MG, Green K, Nielsen KG. Fitness and lung function in children with primary ciliary dyskinesia and cystic fibrosis. Respir Med. 2018 Jun;139:79-85. doi: 10.1016/j.rmed.2018.05.001. Epub 2018 May 3.
citation: Madsen A, Green K, Buchvald F, Hanel B, Nielsen KG. Aerobic fitness in children and young adults with primary ciliary dyskinesia. PLoS One. 2013 Aug 19;8(8):e71409. doi: 10.1371/journal.pone.0071409. eCollection 2013.
citation: Behan L, Rubbo B, Lucas JS, Dunn Galvin A. The patient's experience of primary ciliary dyskinesia: a systematic review. Qual Life Res. 2017 Sep;26(9):2265-2285. doi: 10.1007/s11136-017-1564-y. Epub 2017 Mar 30.
citation: Denizoglu Kulli H, Gurses HN, Zeren M, Ucgun H, Cakir E. Do pulmonary and extrapulmonary features differ among cystic fibrosis, primary ciliary dyskinesia, and healthy children? Pediatr Pulmonol. 2020 Nov;55(11):3067-3073. doi: 10.1002/ppul.25052. Epub 2020 Sep 11.