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Mechanisms of Immune Tolerance and Inflammation in Patients With Cystic Fibrosis With ABPA (ABPA)

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our disclaimer for details. Identifier: NCT00585364
Recruitment Status : Completed
First Posted : January 3, 2008
Last Update Posted : February 15, 2016
National Heart, Lung, and Blood Institute (NHLBI)
Information provided by (Responsible Party):
Jay Kolls, University of Pittsburgh

Brief Summary:
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.

Condition or disease
Cystic Fibrosis Allergic Bronchopulmonary Aspergillosis

Detailed Description:
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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Study Type : Observational
Actual Enrollment : 79 participants
Observational Model: Case-Control
Time Perspective: Prospective
Official Title: SCCOR in Host Factors in Chronic Lung Diseases: Mechanisms of Immune Tolerance and Inflammation in Allergic Bronchopulmonary Aspergillosis (ABPA) in Patients With Cystic Fibrosis
Study Start Date : March 2005
Actual Primary Completion Date : December 2010
Actual Study Completion Date : December 2010

CF (non-ABPA)
cystic fibrosis and culture positive for A. fumigatus in airway cultures.
cystic fibrosis and diagnosis of ABPA
healthy control
healthy non-CF

Primary Outcome Measures :
  1. 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 ]

Secondary Outcome Measures :
  1. 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 ]
  2. 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 ]

Biospecimen Retention:   Samples With DNA
The CF subject's blood will be processed to establish a cell line (lymphocyte transformation) for a source of DNA for future genetic studies.

Information from the National Library of Medicine

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Ages Eligible for Study:   6 Years and older   (Child, Adult, Older Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   Yes
Sampling Method:   Non-Probability Sample
Study Population
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.

Inclusion Criteria:


  • diagnosis of CF
  • age 6 years or older
  • presence of A. fumigatus in culture of airway flora, or the presence of one or more of the diagnostic criteria for ABPA (Control)
  • age and sex matched to CF population

Exclusion Criteria:


  • uncontrolled CF-related diabetes mellitus
  • use of oral steroids at a dose ≥ 0.5 mg/kg/day
  • history of lung transplantation
  • pulmonary exacerbation as defined by requirement for use of intravenous antibiotics or need for hospitalization within the preceding 14 days.
  • patients who have a diagnosis of HIV and have a CD4+ Tcell count below 500 cells/ml will be excluded (control)
  • asthma

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its identifier (NCT number): NCT00585364

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United States, Pennsylvania
Children's Hospital of Pittsburgh of UPMC
Pittsburgh, Pennsylvania, United States, 15213
Sponsors and Collaborators
University of Pittsburgh
National Heart, Lung, and Blood Institute (NHLBI)
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Principal Investigator: Jay K Kolls, MD University of Pittsburgh
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Responsible Party: Jay Kolls, PI, University of Pittsburgh Identifier: NCT00585364    
Other Study ID Numbers: SCCOR
P50HL084932 ( U.S. NIH Grant/Contract )
First Posted: January 3, 2008    Key Record Dates
Last Update Posted: February 15, 2016
Last Verified: February 2016
Individual Participant Data (IPD) Sharing Statement:
Plan to Share IPD: No
Plan Description: This was an observational trial that involved no device or drug.
Keywords provided by Jay Kolls, University of Pittsburgh:
Allergic Bronchopulmonary Aspergillosis
Aspergillus fumigatus
cytokine expression
Additional relevant MeSH terms:
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Pulmonary Aspergillosis
Aspergillosis, Allergic Bronchopulmonary
Cystic Fibrosis
Pathologic Processes
Pancreatic Diseases
Digestive System Diseases
Lung Diseases
Respiratory Tract Diseases
Genetic Diseases, Inborn
Infant, Newborn, Diseases
Lung Diseases, Fungal
Respiratory Hypersensitivity
Respiratory Tract Infections
Hypersensitivity, Immediate
Immune System Diseases