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| ID | Type | Description | Link |
|---|---|---|---|
| P50HL084932 | U.S. NIH Grant/Contract | View source |
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| Name | Class |
|---|---|
| National Heart, Lung, and Blood Institute (NHLBI) | NIH |
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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| CF (non-ABPA) | cystic fibrosis and culture positive for A. fumigatus in airway cultures. | ||
| CF and ABPA | cystic fibrosis and diagnosis of ABPA | ||
| healthy control | healthy non-CF |
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| Measure | Description | Time Frame |
|---|---|---|
| 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. | baseline, 6 month follow-up, ABPA exacerbation |
| Measure | Description | Time Frame |
|---|---|---|
| To test the hypothesis that T cells from CF patients with ABPA will have decreased adaptive regulatory function | 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 |
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Inclusion Criteria:
(CF)
Exclusion Criteria:
(CF)
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Male and female subjects with CF who have A. fumigatus in cultures of airway flora and receive clinical care at the Antonio J. and Janet Palumbo Cystic Fibrosis Center at Children's Hospital of Pittsburgh. Age (± 1 year) and sex matched healthy, non CF controls will also be recruited.
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| Name | Affiliation | Role |
|---|---|---|
| Jay K Kolls, MD | University of Pittsburgh | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Children's Hospital of Pittsburgh of UPMC | Pittsburgh | Pennsylvania | 15213 | United States |
This was an observational trial that involved no device or drug.
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| ID | Term |
|---|---|
| D003550 | Cystic Fibrosis |
| D001229 | Aspergillosis, Allergic Bronchopulmonary |
| ID | Term |
|---|---|
| D010182 | Pancreatic Diseases |
| D004066 | Digestive System Diseases |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
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The CF subject's blood will be processed to establish a cell line (lymphocyte transformation) for a source of DNA for future genetic studies.
| baseline, 6 month follow-up, ABPA exacerbation |
| D030342 |
| Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D007232 | Infant, Newborn, Diseases |
| D055732 | Pulmonary Aspergillosis |
| D001228 | Aspergillosis |
| D009181 | Mycoses |
| D001423 | Bacterial Infections and Mycoses |
| D007239 | Infections |
| D008172 | Lung Diseases, Fungal |
| D012141 | Respiratory Tract Infections |
| D012130 | Respiratory Hypersensitivity |
| D006969 | Hypersensitivity, Immediate |
| D006967 | Hypersensitivity |
| D007154 | Immune System Diseases |