Mounier-Kuhn Syndrome Clinical Trial
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
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.
Status | Recruiting |
Enrollment | 15 |
Est. completion date | April 2019 |
Est. primary completion date | September 2018 |
Accepts healthy volunteers | No |
Gender | All |
Age group | 18 Years to 70 Years |
Eligibility |
Inclusion Criteria: - Has Mounier-Khun Syndrome - Accept and signed informed consent form Exclusion Criteria: - Other morbidity avoiding study procedures |
Country | Name | City | State |
---|---|---|---|
Brazil | Divisao de Pneumologia, Instituto do Coracao, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo | Sao Paulo |
Lead Sponsor | Collaborator |
---|---|
University of Sao Paulo General Hospital |
Brazil,
Type | Measure | Description | Time frame | Safety issue |
---|---|---|---|---|
Primary | Percentage of tracheal-bronchial collapse area before and after applied positive pressure through bronchoscopy | 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 | 18 months | |
Secondary | Apnea+hypopnea (AHI) index in patients with SMK | Study the frequency of OSAHS in patients with SMK; | 24 months | |
Secondary | Percentage of tracheal-bronchial collapse area before and after applied positive pressure measure using chest tomography | Record reversal of collapse with CPAP using chest tomography | 24 months | |
Secondary | Inspiratory and expiratory lung volumes before and after applied positive pressure measure using electrical impedance tomography. | To identify the impact of CPAP on the distribution of pulmonary ventilation through the analysis of electrical impedance tomography. | 24 months |