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

Not Applicable

• Conditions: Mounier-Kuhn Syndrome
• Interventions: Procedure: Non invasive ventilation - Continuous Positive Airway Pressure

• Registration Number: NCT03101059
• Lead Sponsor: 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.

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.

Basic Information
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Recruitment & Eligibility
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Study & Design
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Trial Locations

Study Timeline

• Study First Submitted: 2017-03-23
• Study First Submitted QC: 2017-04-03
• Study First Posted: 2017-04-04
• Study Start: 2017-06-08
• Status Verified: 2017-10
• Last Update Submitted: 2017-10-24
• Last Update Posted: 2017-10-26
• Primary Completion: 2018-09
• Study Completion: 2019-04

Recruitment & Eligibility

• Status: UNKNOWN
• Sex: All
• Target Recruitment: 15

Inclusion Criteria

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

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Exclusion Criteria

• Other morbidity avoiding study procedures

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Study & Design

• Study Type: INTERVENTIONAL
• Study Design: SINGLE_GROUP

Arm && Interventions

Group	Intervention	Description
MKS pax	Non invasive ventilation - Continuous Positive Airway Pressure	Munier-Kuhn Syndrome patients

Primary Outcome Measures

Name	Time	Method
Percentage of tracheal-bronchial collapse area before and after applied positive pressure through bronchoscopy	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

Name	Time	Method
Apnea+hypopnea (AHI) index in patients with SMK	24 months	Study the frequency of OSAHS in patients with SMK;
Percentage of tracheal-bronchial collapse area before and after applied positive pressure measure using chest tomography	24 months	Record reversal of collapse with CPAP using chest tomography
Inspiratory and expiratory lung volumes before and after applied positive pressure measure using electrical impedance tomography.	24 months	To identify the impact of CPAP on the distribution of pulmonary ventilation through the analysis of electrical impedance tomography.

Trial Locations

Locations (1)

Divisao de Pneumologia, Instituto do Coracao, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo; 🇧🇷 Sao Paulo, Brazil