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

Study Status: Not yet recruiting
Recruit Status: Not yet recruiting
Study Type: Interventional

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

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 Summary: 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.
Sponsor: University of Sao Paulo General Hospital

Current Primary Outcome: Percentage of tracheal-bronchial collapse area before and after applied positive pressure through bronchoscopy [ Time Frame: 18 months ]

Original Primary Outcome: Same as current

Current Secondary Outcome:

• Apnea+hypopnea (AHI) index in patients with SMK [ Time Frame: 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 [ Time Frame: 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. [ Time Frame: 24 months ]
  To identify the impact of CPAP on the distribution of pulmonary ventilation through the analysis of electrical impedance tomography.

Original Secondary Outcome: Same as current

Information By: University of Sao Paulo General Hospital

Dates:
Date Received: March 23, 2017
Date Started: April 2017
Date Completion: April 2019
Last Updated: April 3, 2017
Last Verified: March 2017