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Chest Physiotherapy and Lung Function in Primary Ciliary Dyskinesia

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our disclaimer for details. Identifier: NCT01929356
Recruitment Status : Withdrawn (no physiotherapist available for treatment of patients during trial, difficulties with recruitment)
First Posted : August 27, 2013
Last Update Posted : November 24, 2017
Information provided by (Responsible Party):
Universitaire Ziekenhuizen Leuven

Brief Summary:
Primary ciliary dyskinesia (PCD) is a rare disease, caused by impairment of the motile cilia. Patients present with chronic upper and lower respiratory tract infections. The therapy is mainly supportive and based on that of cystic fibrosis. Chest physiotherapy is one of the cornerstones of the therapy, however the influence of chest physiotherapy on lung function (short term and long term) is not clear. For interpretation of longitudinal lung function data it is important to examine the short time effect of chest physiotherapy. We hypothesize that a session of chest physiotherapy improves lung function and that thus lung function tests must be performed in a standardized way.

Condition or disease Intervention/treatment Phase
Primary Ciliary Dyskinesia Procedure: Chest physiotherapy Not Applicable

Detailed Description:

Primary ciliary dyskinesia (PCD) is a rare disease, caused by congenital dysfunction of the motile cilia, located in the upper and lower respiratory tract, in the reproductive system and in the embryonal node. Ineffective ciliary beating results in disturbed mucociliary clearance, which is an important defense mechanism in the respiratory tract. It causes recurrent and chronic upper and lower respiratory tract infections, leading to reversible (mucus plugging) and irreversible lung damage (bronchiectasis, atelectasis, mucus plugging). Just like cystic fibrosis, it is characterized by obstructive lung disease, measured by spirometry.

Using Multiple Breath Washout measurements, it has been shown that peripheral airways disease is present in PCD. Probably, MBW parameters are already abnormal before forced expiratory volume in one second is abnormal.

The therapy for patients with PCD is mainly supportive: regular oral or intravenous antibiotics to treat airway infections and chest physiotherapy to actively increase mucociliary clearance. Chest physiotherapy has the objective to clear mucus from the lungs. Guidelines support the treatment of patients with PCD with chest physiotherapy. It is known that in patients with PCD, exercise has a more bronchodilating effect than the administration of salbutamol. However, no objective data describe the acute effect of chest physiotherapy on spirometry and MBW parameters. Moreover, short term effects of intervention on spirometry and MBW parameters can influence the interpretation of long-term evolution of these parameters.

In cystic fibrosis, one study has assessed the short-term influence of chest physiotherapy on lung function, measured by spirometry and MBW before and after chest physiotherapy. The authors found no significant influence of chest physiotherapy on spirometry parameters, nor on MBW parameters. Therefore, longitudinal interpretation can be performed irrespective of the timing of the lung function measurements. However, cystic fibrosis and PCD have a different pathophysiological mechanism with a more 'mechanical' disturbance of the mucociliary clearance in PCD, compared to a more 'viscous' disturbance of mucociliary clearance in CF. Therefore, the results in CF are not applicable to PCD. Even more, we hypothesize that chest physiotherapy will have a significant effect on lung function parameters (spirometry and MBW) in patients with PCD due to its external mechanical effect on mucus clearance.

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Study Type : Interventional  (Clinical Trial)
Actual Enrollment : 0 participants
Allocation: N/A
Intervention Model: Single Group Assignment
Masking: None (Open Label)
Primary Purpose: Treatment
Official Title: The Influence of Chest Physiotherapy on Lung Function Parameters in Primary Ciliary Dyskinesia
Estimated Study Start Date : October 2013
Actual Primary Completion Date : November 2017
Actual Study Completion Date : November 2017

Resource links provided by the National Library of Medicine

Arm Intervention/treatment
Experimental: chest physiotherapy
session of 20 minutes chest physiotherapy with physiotherapist, use of airway clearance techniques, PEP (positive expiratory pressure) device
Procedure: Chest physiotherapy
20 minutes of chest physiotherapy by physiotherapist

Primary Outcome Measures :
  1. Difference in FEV1 before and after treatment [ Time Frame: 30 minutes ]
    Lung function will be performed before a session of chest physiotherapy, and repeated 30 minutes after a chest physiotherapy session of 20 minutes with airway clearance techniques and use of PEP mask

Secondary Outcome Measures :
  1. Difference in LCI before and after chest physiotherapy [ Time Frame: 30 minutes ]
    Multiple Breath Washout (MBW) will be performed before and 30 min after a session of chest physiotherapy. The difference in LCI (lung clearance index) will be used as secondary outcome.

Information from the National Library of Medicine

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Ages Eligible for Study:   6 Years to 50 Years   (Child, Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No

Inclusion Criteria:

  • Primary ciliary dyskinesia
  • able to perform spirometry and MBW (>6 years)

Exclusion Criteria:

  • acute exacerbation

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its identifier (NCT number): NCT01929356

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University Hospital Gasthuisberg Leuven
Leuven, Belgium, 3000
Sponsors and Collaborators
Universitaire Ziekenhuizen Leuven
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Principal Investigator: Mieke Boon, MD research fellow
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Responsible Party: Universitaire Ziekenhuizen Leuven Identifier: NCT01929356    
Other Study ID Numbers: UZLMB001
First Posted: August 27, 2013    Key Record Dates
Last Update Posted: November 24, 2017
Last Verified: August 2013
Keywords provided by Universitaire Ziekenhuizen Leuven:
lung function tests
chest physiotherapy
Additional relevant MeSH terms:
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Ciliary Motility Disorders
Movement Disorders
Central Nervous System Diseases
Nervous System Diseases
Neurologic Manifestations
Respiratory Tract Diseases
Otorhinolaryngologic Diseases
Abnormalities, Multiple
Congenital Abnormalities
Genetic Diseases, Inborn