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Airway Collapse in Patients With Mounier-Kuhn Syndrome: Titration With Positive Pressure to Reduce Collapse

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ClinicalTrials.gov Identifier: NCT03101059
Recruitment Status : Unknown
Verified October 2017 by Rafael Stelmach, University of Sao Paulo General Hospital.
Recruitment status was:  Recruiting
First Posted : April 4, 2017
Last Update Posted : October 26, 2017
Sponsor:
Information provided by (Responsible Party):
Rafael Stelmach, University of Sao Paulo General Hospital

Brief Summary:
Mounier-Kuhn syndrome (MKS), or congenital tracheobronchiomegaly, is an entity characterized by dilation of the trachea and bronchi, associated with respiratory infections.The main signs and symptoms are cough, bulging and purulent expectoration, digital clubbing, dyspnoea, and wheezing.Some of these symptoms are believed to be due to excessive collapse of the intra-thoracic trachea and bronchi, resulting in airways obstruction of more than 50% . The purpose of this study is to identify and reduce tracheal collapse.

Condition or disease Intervention/treatment Phase
Mounier-Kuhn Syndrome Procedure: Non invasive ventilation - Continuous Positive Airway Pressure Not Applicable

Detailed Description:
Mounier-Kuhn syndrome (MKS), or congenital tracheobronchiomegaly, is an entity characterized by dilation of the trachea and bronchi, associated with respiratory infections. The prevalence among patients with respiratory symptoms is 0.4 to 1.6%. Its histological features include the atrophy or absence of longitudinal elastic fibers and smooth muscle cells of the airways, responsible for the structural alterations found, such as tracheobronchiomegaly, the presence of inter cartilaginous diverticula, bulging and dilation of the walls of the trachea and bronchi. The main signs and symptoms are cough, bulging and purulent expectoration, digital clubbing, dyspnoea, and wheezing and wheezing accompanied by recurrent respiratory infection. There may be association with other comorbidities such as gastroesophageal reflux disease, chronic obstructive pulmonary disease, bronchiectasis and obstructive sleep apnea / hypopnea syndrome (OSAS). Some of these symptoms are believed to be due to the tracheobronchial disease present in some patients, defined by excessive collapse of the intrathoracic trachea and bronchi resulting in airways obstruction of more than 50%. The main clinical impact is obstruction to expiratory airflow, with consequent air entrapment, reduction of cough and bronchial hygiene effectiveness, facilitating recurrent respiratory infections. Because it is a rare morbidity and little studied, the specific therapy is not consensual, and the main interventions are extrapolated from other pathologies. The use of non invasive mechanical ventilation (NIMV) with continuous positive airway pressure (CPAP) is reported as an option for treatment, however, there are no randomized studies proving its efficacy. The purpose of this study is to identify and reduce tracheal collapse and bronchi of SMK carriers with the use of positive pressure (CPAP-NIV) and to analyse their repercussion in the small airways.

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Study Type : Interventional  (Clinical Trial)
Estimated Enrollment : 15 participants
Allocation: N/A
Intervention Model: Single Group Assignment
Masking: None (Open Label)
Primary Purpose: Supportive Care
Official Title: Evaluation of the Presence of Airway Collapse in Patients With Mounier-Kuhn Syndrome and Titration With Continuous Positive Pressure Through Non-invasive Mechanical Ventilation to Reduce Collapse
Actual Study Start Date : June 8, 2017
Estimated Primary Completion Date : September 2018
Estimated Study Completion Date : April 2019

Resource links provided by the National Library of Medicine

MedlinePlus related topics: Shock

Arm Intervention/treatment
Experimental: MKS pax
Munier-Kuhn Syndrome patients
Procedure: Non invasive ventilation - Continuous Positive Airway Pressure
To identify, through bronchoscopy, the prevalence of collapse and whether it is possible to counteract an optimum pressure generated by NIV with CPAP that reduces tracheal and bronchial collapse in patients with SMK; To study the frequency of OSAS in patients with MKS ; Record reversal of collapse with CPAP using chest tomography; To identify the impact of CPAP on the distribution of pulmonary ventilation through the analysis of electrical impedance tomography.




Primary Outcome Measures :
  1. Percentage of tracheal-bronchial collapse area before and after applied positive pressure through bronchoscopy [ Time Frame: 18 months ]
    Identify the prevalence of collapse and whether it is possible to counteract an optimum pressure generated by NIV with CPAP that reduces tracheal and bronchial collapse in patients with SMK


Secondary Outcome Measures :
  1. Apnea+hypopnea (AHI) index in patients with SMK [ Time Frame: 24 months ]
    Study the frequency of OSAHS in patients with SMK;

  2. Percentage of tracheal-bronchial collapse area before and after applied positive pressure measure using chest tomography [ Time Frame: 24 months ]
    Record reversal of collapse with CPAP using chest tomography

  3. Inspiratory and expiratory lung volumes before and after applied positive pressure measure using electrical impedance tomography. [ Time Frame: 24 months ]
    To identify the impact of CPAP on the distribution of pulmonary ventilation through the analysis of electrical impedance tomography.



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Ages Eligible for Study:   18 Years to 70 Years   (Adult, Older Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
Criteria

Inclusion Criteria:

  • Has Mounier-Khun Syndrome
  • Accept and signed informed consent form

Exclusion Criteria:

  • Other morbidity avoiding study procedures

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 ClinicalTrials.gov identifier (NCT number): NCT03101059


Contacts
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Contact: Rafael Stelmach, MD-PHD 551126615191 rafael.stelmach@incor.usp.br
Contact: Evelise Lima, MD 551126615191 eveliselima53@gmail.com

Locations
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Brazil
Divisao de Pneumologia, Instituto do Coracao, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo Recruiting
Sao Paulo, Brazil, 05403000
Contact: Rafael Stelmach, MD-PHD    551126615191    rafael.stelmach@incor.usp.br   
Contact: Evelise Lima, MD    551126615191    eveliselima53@gmail.com   
Sponsors and Collaborators
University of Sao Paulo General Hospital
Publications automatically indexed to this study by ClinicalTrials.gov Identifier (NCT Number):
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Responsible Party: Rafael Stelmach, Clinical Professor, University of Sao Paulo General Hospital
ClinicalTrials.gov Identifier: NCT03101059    
Other Study ID Numbers: CAAE64001317400000068
First Posted: April 4, 2017    Key Record Dates
Last Update Posted: October 26, 2017
Last Verified: October 2017
Individual Participant Data (IPD) Sharing Statement:
Plan to Share IPD: No

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Studies a U.S. FDA-regulated Drug Product: No
Studies a U.S. FDA-regulated Device Product: No
Keywords provided by Rafael Stelmach, University of Sao Paulo General Hospital:
Tracheal
Bronchial
Tracheobronchiomegaly
Collapse
obstructive sleep apnea syndrome
Computer Tomography
Impedance Tomography
Additional relevant MeSH terms:
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Tracheobronchomegaly
Syndrome
Shock
Disease
Pathologic Processes
Bronchial Diseases
Respiratory Tract Diseases
Respiratory System Abnormalities
Tracheal Diseases
Congenital Abnormalities