Immotile Cilia Syndrome, Kartagener Syndrome, Primary Ciliary Dyskinesia
Conditions
Keywords
Primary Ciliary Dyskinesia, Kartagener Syndrome, Immotile Cilia Syndrome, Pulmonary Function, Respiratory Muscle Strength, Exercise Capacity, Physical Fitness, Inspiratory Muscle Strength Training
Brief summary
Decreased pulmonary function, peripheral muscle strength, and exercise capacity were reported in primary ciliary dyskinesia (PCD) in recent studies. We aimed to investigate the data conducted between 10 July 2015 and 10 January 2015 of pulmonary function, respiratory muscle strength, exercise capacity, physical fitness, and activities of daily living (ADL) in PCD and healthy counterparts retrospectively at the first stage of the study and the effects of inspiratory muscle training on pulmonary function, respiratory muscle strength, and exercise capacity in PCD patients with decreased inspiratory muscle strength from the database recorded between 10 July 2015 and 10 January 2015 retrospectively at the second stage of the study.
Interventions
OTHERData Collection
The first stage of the study: Retrospective data collection of pulmonary function, respiratory muscle strength, exercise capacity, physical fitness, and activities of daily living (ADL). The second stage of the study -Retrospective data of pulmonary function, respiratory muscle function, functional capacity before and after supervised inspiratory muscle training with POWERbreathe K5 device using tapered flow resistive loading and airway clearance techniques performed for 6 weeks in PCD with decreased respiratory muscle strength.
Sponsors
Hacettepe University
Study design
Observational model
COHORT
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
8 Years to 18 Years
Healthy volunteers
Yes
Inclusion criteria
* Clinically stable * Being able to cooperate with the assessments * Not having any orthopedic, or neurological problems
Exclusion criteria
* Not being clinically stable * Not being able to cooperate with the assessments * Having any orthopedic, or neurological problems
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| A primer outcome for the first stage of the study: Physical fitness | 10 July 2015-10 January 2015-retrospectively | Munich Physical Fitness Test (MFT) was used to evaluate physical fitness. It is a test with six parameters including ball bounce, sandbag throwing, bending, vertical jumping, hanging and step test. |
| A primer outcome for the second stage of the study: Functional capacity | 10 July 2015-10 January 2015-retrospectively | Functional capacity was evaluated with six minute walk test (6MWT). |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Respiratory muscle functions | 10 July 2015-10 January 2015-retrospectively | Respiratory muscle strength (maximal inspiratory pressure (MIP);maximal expiratory pressure (MEP), respectively) was measured with a portable, electronic mouth pressure monitor (Micro Medical MicroMPM, UK) and recorded. For second stage of the study; average inspiratory flow, inspiratory volume, and inspiratory work was evaluated based on the outcomes of the Breathe-Link software using POWERbreathe K5 device before and after IMT with tapered flow resistive loading and recorded. |
| Demographic features | 10 July 2015-10 January 2015-retrospectively | The age was recorded in years. |
| Activities of Daily Living | 10 July 2015-10 January 2015-retrospectively | Glittre activities of daily living (ADL) test was used to evaluate daily life activity. |
| Exercise Capacity | 10 July 2015-10 January 2015-retrospectively | Exercise capacity was assessed with the modified shuttle walk test. |
| Pulmonary Function | 10 July 2015-10 January 2015-retrospectively | Pulmonary function (FEV1 (forced expiratory volume in one second), FVC (forced vital capacity), FEV1/FVC, FEF25-75 (%) (forced expiratory volume 25-75 %) and PEF (peak expiratory flow)) assessed with a portable spirometer (Spirobank MIR, Italy). |
Countries
Turkey (Türkiye)
Outcome results
None listed