Bronchial Inflammation in Patients With Bronchiolithis Obliterans

Study Overview
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Brief Summary
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The investigators plan to study the variability of lung function parameters and bronchial inflammatory markers in 20 patients with bronchiolitis obliterans at the age of 6 to 25 years compared with an age- and sex-matched control group. The investigators will perform a pulmonary function test (body plethysmography with TLCO), and measure the fraction of exhaled nitric oxide (eNO). Further a blood sample is drawn to determine the systemic inflammation. Finally induced sputum is collected and a cell count is performed, and cells and supernatants are analyzed for inflammatory markers. This investigation will be repeated after 4-6 weeks. The aim of this study is to define baseline values and the variability of possible outcome parameters for future interventional studies.

Detailed Description
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The purpose of this study is to characterize the variability of lung function values and bronchial inflammation of 20 Patients with Bronchiolitis obliterans aged between 6 up to 25 years. The patients' data will be compared with the results of a reference group with similar age conditions to determine possible outcome-parameters for following interventional studies. The sputum samples will be processed and quantitatively analyzed to get a profile of the cytological composition. (Sputum and serum samples are analyzed by quantitative real-time polymerase chain reaction (PCR) (qRT-PCR) and by cytometric bead assay (CBA).) Methods and Work Programme: This study consists of two study visits (V1 and V2) V1: Measurement of nitric oxide in expired air (eNO) Lung function testing with spirometry and body plethysmography Bronchodilation Lung function testing with spirometry and body plethysmography Blood test: blood count, CRP, RAST, serum inflammatory mediators, (genetic markers of the non-specific pulmonary defense system) Induced sputum for inflammatory mediators and microbiological investigations V2: Measurement of nitric oxide in expired air (eNO) Lung function testing with spirometry and body plethysmography Bronchodilation Lung function testing with spirometry and body plethysmography Induced sputum for inflammatory mediators and microbiological investigations

Official Title
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Lung Function and Bronchial Inflammation in Patients With Bronchiolithis Obliterans

Conditions
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Bronchiolitis Obliterans

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: informed consent between 6 and 25 years of age Known Bronchiolitis obliterans no Bronchiolitis obliterans(depending on the study group) Ability to perform lung function tests and inhalation Exclusion Criteria: Acute illness with systemic or bronchial inflammation every chronic condition or infection (eg HIV, tuberculosis, malignancy) pregnancy known alcohol and/ or drug abuse Inability to understand the extent and scope of the study Participation in another study

Ages Eligible for Study
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Minimum Age: 6 Years
Maximum Age: 25 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Subject-Group<br>Patients suffering from doctors diagnosed bronchiolitis obliterans | |
| Control Group<br>age- and sex matched to subject-group | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Change of FEF75 over time | Look for changes/ variability of this lung function parameter | V1: Day 1 - V2: Day 28-42 | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| change of sputum cell count over time | induced sputum will be analyzed for different cellular components such as alveolar macrophages, neutrophils, lymphocytes and others. Epithelial cells will be excluded | V1: Day 1 - V2: Day 28-42 | 
| change of low CrP over time | serum parameter for systemic inflammation | V1: Day 1 - V2: Day 28-42 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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bronchial inflammation, Bronchiolitis obliterans, Sputum, Blood, Inflammation, adaptive immune system, innate immune system