The investigators here compare lung function parameters (RV, RV/TLC and FEF75) with the results of the Lung Clearance index (LCI) . Further this study evaluates bronchial inflammatory markers in 20 patients with bronchiolitis obliterans at the age of 6 to 28 years compared with an age- and sex-matched control group. The investigators will perform a pulmonary function test (body plethysmography with DLCO) and compare the results with the LCI. Further we will measure the fraction of exhaled nitric oxide (FeNO) and draw a blood sample to determine the level of systemic inflammation. Finally induced sputum is collected and a cell count is performed, and cells and supernatants are analyzed for inflammatory markers. This study will set baseline markers for future interventional studies.
The purpose of this study is to compare lung function values with the Lung clearance index of 20 Patients with Bronchiolitis obliterans aged between 6 up to 28 years. Further we aim to compare the bronchial Inflammation between the patients and 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: * Measurement of nitric oxide in expired air (FeNO) * Lung function testing with spirometry and body plethysmography * Lung clearance index (LCI) * Bronchodilation * 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
Study Type
OBSERVATIONAL
Enrollment
37
Children's Hospital, Goethe-University
Frankfurt am Main, Hesse, Germany
LCI compared to RV/TLC%-pred.
Correlation of these lung function Parameters in patients with BO compared to healthy controls
Time frame: during Visit 1 (single day, single observation)
change of sputum cell count over time (percentage of neutrophils)
induced sputum will be analyzed for different cellular components such as alveolar macrophages, neutrophils, lymphocytes and others. Epithelial cells will be excluded
Time frame: during Visit 1 (single day, single observation)
Comparison of low CrP
serum parameter for systemic inflammation
Time frame: during Visit 1 (single day, single observation)
FVC (%-pred.)
To compare FVC (%-pred.)of patients suffering from BO with FVC (%-pred.)of a healthy control group
Time frame: during Visit 1 (single day, single observation)
FEV1 (%-pred.)
To compare FEV1 (%-pred.)of patients suffering from BO withFEV1 (%-pred.)of a healthy control group
Time frame: during Visit 1 (single day, single observation)
Tiffeneau-Index
To compare Tiffeneau-Index of patients suffering from BO with Tiffeneau-Index of a healthy control group
Time frame: during Visit 1 (single day, single observation)
sRtot (%-pred.)
To compare sRtot (%-pred.) of patients suffering from BO with sRtot (%-pred.) of a healthy control group
Time frame: during Visit 1 (single day, single observation)
RV/TLC (%-pred.)
To compare RV/TLC (%-pred.) of patients suffering from BO with RV/TLC (%-pred.) of a healthy control group
Time frame: during Visit 1 (single day, single observation)
IL-6 (pg/ml)
comparing IL-6 in Serum of patients with BO with healthy controls
Time frame: during Visit 1 (single day, single observation)
IL-8 (pg/ml)
comparing IL-8 in Serum of patients with BO with healthy controls
Time frame: during Visit 1 (single day, single observation)1
IL-17 (pg/ml)
comparing IL-17 in Serum of patients with BO with healthy controls
Time frame: during Visit 1 (single day, single observation)
IL-6 (mRNA delta-delta-ct)
compare IL-6 mRNA in Sputum cells of patients with BO with healthy controls
Time frame: during Visit 1 (single day, single observation)
IL-8 (mRNA delta-delta-ct)
compare IL-8 mRNA in Sputum cells of patients with BO with healthy controls
Time frame: during Visit 1
IL-17 (mRNA delta-delta-ct)
compare IL-17 mRNA in Sputum cells of patients with BO with healthy controls
Time frame: during Visit 1 (single day, single observation)
Percentage of FOXP3 positive cells in Sputum
compare percentage of FOXP3 positive cells in Sputum cells of patients with BO with healthy controls
Time frame: during Visit 1 (single day, single observation)
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