Physical Fitness, Exercise Capacity and Activities of Daily Living in Primary Ciliary Dyskinesia: a Retrospective Study

Completed

Conditions: Primary Ciliary Dyskinesia
Immotile Cilia Syndrome
Kartagener Syndrome

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.

Recruitment & Eligibility

Inclusion Criteria

Exclusion Criteria

Arm && Interventions

Group	Intervention	Description
Primary Ciliary Dyskinesia (PCD)	Data Collection	The first stage of the study: Data of 20 PCD patients from the database recorded between 10 July 2015 and 10 January 2017. The second stage of the study: -Data of 14 PCD patients who had lower inspiratory muscle strength from the same database underwent supervised inspiratory muscle training (IMT) with tapered flow resistive loading using Powerbreathe K5 device at our research unit and airway clearance techniques (ACT) as a home-based therapy for 6 weeks from the database recorded between 10 July 2015 and 10 January 2017.
Healthy Group	Data Collection	Data of 20 healthy subjects from the database recorded between 10 July 2015 and 10 January 2017.

Primary Outcome Measures

Name	Time	Method
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).
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.

Secondary Outcome Measures

Name	Time	Method
Demographic features	10 July 2015-10 January 2015-retrospectively	Body mass index was calculated by dividing the weight by the square of the height (kg/m\^2) and recorded.
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).
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.
Exercise Capacity	10 July 2015-10 January 2015-retrospectively	Exercise capacity was assessed with the modified shuttle walk test.
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.