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

NCT00585364 | Completed

• 2 other identifiers: SCCORP50HL084932
• Study Type: observational
• Target: 79
• Locations: 1 country
• Sites: 1

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.

Conditions

Cystic Fibrosis; Allergic Bronchopulmonary Aspergillosis

Keywords

Allergic Bronchopulmonary Aspergillosis; Aspergillus fumigatus; cytokine expression

Outcome Measures

Primary Outcomes (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.

  baseline, 6 month follow-up, ABPA exacerbation

Secondary Outcomes (2)

• 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

  baseline, 6 month follow-up, ABPA exacerbation

Study Arms (3)

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

Eligibility Criteria

• Age: 6 Years+
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• 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.

You may qualify if:

• (CF)
• 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

You may not qualify if:

• (CF)
• 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

Sponsors & Collaborators

• University of Pittsburgh: lead
• National Heart, Lung, and Blood Institute (NHLBI): collaborator

Study Sites (1)

Children's Hospital of Pittsburgh of UPMC

Pittsburgh, Pennsylvania, 15213, United States

Location

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.

MeSH Terms

Conditions

Cystic Fibrosis; Aspergillosis, Allergic Bronchopulmonary

Condition Hierarchy (Ancestors)

Pancreatic Diseases; Digestive System Diseases; Lung Diseases; Respiratory Tract Diseases; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Infant, Newborn, Diseases; Pulmonary Aspergillosis; Aspergillosis; Mycoses; Bacterial Infections and Mycoses; Infections; Lung Diseases, Fungal; Respiratory Tract Infections; Respiratory Hypersensitivity; Hypersensitivity, Immediate; Hypersensitivity; Immune System Diseases

Study Officials

• Jay K Kolls, MD

  University of Pittsburgh

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: CASE CONTROL
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: PI

Study Record Dates

• First Submitted: January 1, 2008
• First Posted: January 3, 2008
• Study Start: March 1, 2005
• Primary Completion: December 1, 2010
• Study Completion: December 1, 2010
• Last Updated: February 15, 2016

Record last verified: 2016-02

Data Sharing

• IPD Sharing: Will not share

Locations

US (1)