The Role of Bacteria and Genetic Variations in Cystic Fibrosis
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Purpose
This study will examine 1) the role of hereditary factors in cystic fibrosis; i.e., the relationship of the disease to specific gene variations, and 2) the role of bacterial products involved in lung infections substances produced by bacteria may worsen the disease.
Patients with cystic fibrosis who are being followed by the Medical College of Wisconsin or the University of Wisconsin-Madison are eligible for this study. Participants will have blood tests, pulmonary function tests, a sputum culture, and buccal swabbing (cotton swabbing of the inside of the cheek to collect cells for DNA study). In addition, their medical records will be reviewed for a history of lung infections and the results of various tests, including pulmonary function studies, chest X-rays and bacterial cultures. Blood samples collected previously at the Medical College of Wisconsin or the University of Wisconsin-Madison will also be analyzed for antibodies to bacteria.
Although this is a one-time study, participants may be asked to return for repeated tests.
| Condition |
|---|
| Lung Diseases |
| Study Type: | Observational |
| Official Title: | Clinical Course in Cystic Fibrosis: The Effects of Pseudomonas Aeruginosa and Potential Modifier Genes |
| Estimated Enrollment: | 200 |
| Study Start Date: | June 20, 2001 |
Individuals with cystic fibrosis (CF) are susceptible to chronic bacterial colonization by Pseudomonas aeruginosa, which results in deterioration of lung function and, eventually, death. In this study, we hope to improve our understanding of the innate immune response to infection by strains of P. aeruginosa that express type III cytotoxins and to delineate better the role of modifier genes in disease progression.
We will examine relationships between the patient's clinical course, the presence of antibodies to P. aeruginosa, and single nucleotide polymorphisms in suspected CF modifier genes.
Eligibility
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the contacts provided below. For general information, Learn About Clinical Studies.
| Ages Eligible for Study: | 9 Years to 80 Years (Child, Adult, Senior) |
| Sexes Eligible for Study: | All |
| Accepts Healthy Volunteers: | No |
- INCLUSION CRITERIA:
Patients with cystic fibrosis who have a defined mutation in CFTR (e.g., any of the known variants of the CFTR gene, such as the delta F508 allele) born in the state of Wisconsin since 1985 or otherwise followed by the cystic fibrosis centers at the Medical College of Wisconsin or University of Wisconsin-Madison.
Patients will have been tested or will be tested for the CFTR gene under another protocol (96-H-0100).
Patients may be colonized with P. aeruginosa or other organisms (e.g., Burkholderia cepacia).
The age range of NIH participants in this study is from 9 to 80 years old.
EXCLUSION CRITERIA:
There are no exclusion criteria.
Contacts and Locations
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 ClinicalTrials.gov identifier (NCT number): NCT00043225
| Contact: Mary Haughey, R.N. | (301) 496-3632 | mhaughey@nhlbi.nih.gov | |
| Contact: Joel Moss, M.D. | (301) 496-1597 | mossj@nhlbi.nih.gov |
| United States, Maryland | |
| National Institutes of Health Clinical Center, 9000 Rockville Pike | Recruiting |
| Bethesda, Maryland, United States, 20892 | |
| Contact: For more information at the NIH Clinical Center contact Patient Recruitment and Public Liaison Office (PRPL) 800-411-1222 ext TTY8664111010 prpl@mail.cc.nih.gov | |
| United States, Washington | |
| University of Washington | Recruiting |
| Seattle, Washington, United States, 98195 | |
| United States, Wisconsin | |
| University of Wisconsin | Recruiting |
| Madison, Wisconsin, United States, 53792 | |
| Medical College of Wisconsin | Recruiting |
| Milwaukee, Wisconsin, United States | |
| Principal Investigator: | Joel Moss, M.D. | National Heart, Lung, and Blood Institute (NHLBI) |
More Information
Additional Information:
Publications:
| Responsible Party: | National Heart, Lung, and Blood Institute (NHLBI) |
| ClinicalTrials.gov Identifier: | NCT00043225 History of Changes |
| Other Study ID Numbers: |
010198 01-H-0198 |
| First Submitted: | July 13, 2006 |
| First Posted: | August 7, 2002 |
| Last Update Posted: | October 19, 2017 |
| Last Verified: | August 9, 2017 |
Keywords provided by National Institutes of Health Clinical Center (CC) ( National Heart, Lung, and Blood Institute (NHLBI) ):
|
Type III Secretion Pathways Exotoxin A Polymorphisms Cystic Fibrosis and Pseudomonas Aeruginosa Adenosine Deaminase Deficiency |
Severe Combined Immune Deficiency SCID ADA-SCID Immune Deficiency |
Additional relevant MeSH terms:
|
Fibrosis Cystic Fibrosis Lung Diseases Pathologic Processes Pancreatic Diseases |
Digestive System Diseases Respiratory Tract Diseases Genetic Diseases, Inborn Infant, Newborn, Diseases |