The goal of this study is to identify the immunological factors that influence a patient's response to the presence of the fungus Aspergillus fumigatus (A. fumigatus) in the lungs. In patients with cystic fibrosis (CF), this fungus is not known to cause damage to the lungs, but some patients respond with an allergic reaction that may cause wheeze, cough, or difficulty breathing. Approximately 230 patients will be enrolled with an additional 60 people who do not have CF and who do not have a history of asthma to serve as a comparison group.
In this cohort the investigators will study blood T-cell responses to Aspergillus antigens to determine if patients with ABPA have greater type 2 responses as measured by IL-13 compared to CF or non-CF controls.
Study Type
OBSERVATIONAL
Enrollment
79
Children's Hospital of Pittsburgh of UPMC
Pittsburgh, Pennsylvania, United States
To test the hypothesis that the white blood cells of CF patients with ABPA will demonstrate increased inflammatory cytokine expression in response to binding of A. fumigatus antigens compared to white blood cells from non-ABPA patients.
Time frame: baseline, 6 month follow-up, ABPA exacerbation
To test the hypothesis that T cells from CF patients with ABPA will have decreased adaptive regulatory function
Time frame: baseline, 6 month follow-up, ABPA exacerbation
To test the hypothesis that surface-bound TGF beta is critical for the development and maintenance of immune tolerance to A. fumigatus antigens
Time frame: baseline, 6 month follow-up, ABPA exacerbation
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