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

Hacettepe University48 enrolled

Overview

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.

Study Type

OBSERVATIONAL

Enrollment

Conditions

Primary Ciliary Dyskinesia Kartagener Syndrome Immotile Cilia Syndrome

Interventions

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.

Eligibility

Sex: ALLMin age: 8 YearsMax age: 18 YearsHealthy volunteers:

Medical Language ↔ Plain English

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

Outcomes

Primary Outcomes

A primer outcome for the first stage of the study: Physical fitness

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.