Natural History of Bronchiectasis

This study is currently recruiting participants. (see Contacts and Locations)
Verified December 2013 by National Institutes of Health Clinical Center (CC)
Sponsor:
Information provided by:
National Institutes of Health Clinical Center (CC)
ClinicalTrials.gov Identifier:
NCT00943514
First received: July 21, 2009
Last updated: June 24, 2014
Last verified: December 2013
  Purpose

Background:

  • Bronchiectasis is a disease characterized by airways that are inflamed, abnormally dilated, and chronically infected. Individuals with bronchiectasis have a history of chronic and recurring respiratory infections. Depending on the underlying cause, these infections may involve the entire respiratory tract, resulting in sinus, ear, and lung disease.
  • Bronchiectasis continues to be a significant problem in developing countries and in specific groups of individuals, particularly in people who have cystic fibrosis. Although treatments are available or under development for bronchiectasis related to cystic fibrosis, many of the disease-specific treatments may not be effective for bronchiectasis not related to cystic fibrosis.

Objectives:

- To study the natural history of bronchiectasis to identify inherited and immune factors that may explain why certain individuals have chronic recurring infections.

Eligibility:

  • Individuals 5 years of age and older who have an established diagnosis of bronchiectasis or a history of chronic/recurring respiratory infections.
  • Direct family members (e.g., parents, siblings, children) of patients in the study may also be asked to participate.

Design:

  • Potential participants will be screened with an initial clinic evaluation and full medical history, as well as a general quality of life and respiratory symptom questionnaire.
  • The following standard procedures may be done as part of the study: air sampling from the nose; imaging studies, which may include an x-ray or computed tomography (CT), lung function tests; and collection of samples of blood, urine, and sputum (phlegm or mucus). Other tests may be performed as required by the researchers, and will be explained to patients as needed.
  • Both patients and relatives (if asked to participate) will provide the following samples: blood or buccal (cheek swab) cells for genetic testing, sputum, and urine.
  • To prevent infections and potential disease progression, patients may receive standard medical care and treatment for bronchiectasis and related infections during this protocol.
  • Patients older than 18 years of age will be invited to join the Bronchiectasis Research Registry, a program supported by the Chronic Obstructive Pulmonary Disease (COPD) Foundation. The purpose of the program is to identify a group of people who are interested in receiving information about research studies focused on bronchiectasis and possibly participating in these research studies. Participants may be offered the opportunity to participate in selected clinic trials related to bronchiectasis or related medical conditions.

Condition
Bronchiectasis
Cystic Fibrosis
Autoimmune Disease
Common Variable Immunodeficiency

Study Type: Observational
Study Design: Time Perspective: Prospective
Official Title: Natural History of Bronchiectasis

Resource links provided by NLM:


Further study details as provided by National Institutes of Health Clinical Center (CC):

Estimated Enrollment: 300
Study Start Date: July 2009
Detailed Description:

Bronchiectasis, or abnormal dilation of the airways, is a condition typically characterized by chronic and recurring respiratory tract infections. Frequently, depending on the underlying cause, these infections involve the entire respiratory tract resulting in sinus, ear, and lung disease. This condition used to be more common in children prior to immunizations for childhood infections. It continues to be a significant problem in developing countries and in specific groups of individuals in the U.S. Cystic fibrosis (CF) is the most commonly associated genetic condition and tremendous strides have been made in recent years in understanding the mechanisms of this disease that are leading to a multitude of emerging novel treatment strategies. The mechanisms of other causes for bronchiectasis have not evolved to this degree, and many of the disease-specific treatments being assessed for cystic fibrosis may not be effective for non-CF bronchiectasis.

This protocol is aimed at exploring associated causes and mechanisms for the development of bronchiectasis in patients with chronic and recurring respiratory tract infections. Participants will undergo testing based on their individual clinical presentations to look for known associated conditions. Additionally, some patients may be followed over time to better understand the natural history of evolving bronchiectasis and to assess the effect of management strategies on the course of the disease. As potential novel therapies develop, participants may be offered the opportunity to participate in clinical treatment trials.

  Eligibility

Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   No
Criteria
  • PARTICIPANT INCLUSION CRITERIA:

    1. Children age 5 years and above and adults referred to the NIH with chronic or recurring respiratory infections will be eligible for participation.
    2. Males and females will be considered without regard to race or ethnicity or upper age limit.
    3. Emphasis will be primarily on non CF bronchiectasis for elucidation of mechanisms of infection susceptibility. However, select patients with cystic fibrosis or acquired immune defects (such as HIV) may be studied if relevant host defects are suspected or if needed for comparison purposes.
    4. Subjects must have a primary or referring physician
    5. Subjects must be willing to have samples stored

INCLUSION CRITERIA FOR RELATIVES:

Male and female relatives will be accepted without limitation due to age.

PARTICIPANT EXCLUSION CRITERIA:

  1. We wish to avoid enrolling subjects, especially children, who have common respiratory problems (aeroallergen sensitivity, asthma, gastric esophageal reflux) that are not associated with an underlying abnormality in host defenses. Evidence of significance of chronic or recurring infections suggestive of an underlying airway surface or systemic host defense defect should be documented by one or more of the following: a) sinus or lower airway cultures positive for bacterial, fungal, or mycobacterial pathogens characteristic of these defects; or b) radiographic evidence of sinusitis with mucosal thickening and/or air-fluid levels; or c) radiographic evidence of bronchiectasis; d) severity of otitis media requiring placement of tympanic membrane pressure equalization tubes; e) severity of sinusitis requiring surgical intervention.
  2. Patients who are unable or unwilling to provide informed consent either directly or via appropriately designated surrogate.
  3. Any patient who, in the opinion of the Investigator, is unable or unwilling to comply with regular follow-up or is unlikely to provide pertinent information regarding disease progression or response to treatment may be excluded from longitudinal follow-up.
  4. Children under the age of 5 will be excluded from this protocol.
  Contacts and Locations
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, see Learn About Clinical Studies.

Please refer to this study by its ClinicalTrials.gov identifier: NCT00943514

Contacts
Contact: Reginald J Claypool, R.N. (301) 402-7831 rclaypool@niaid.nih.gov

Locations
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   
Sponsors and Collaborators
Investigators
Principal Investigator: Kenneth N Olivier, M.D. National Heart, Lung, and Blood Institute (NHLBI)
  More Information

Additional Information:
Publications:
ClinicalTrials.gov Identifier: NCT00943514     History of Changes
Other Study ID Numbers: 090172, 09-I-0172
Study First Received: July 21, 2009
Last Updated: June 24, 2014
Health Authority: United States: Federal Government

Keywords provided by National Institutes of Health Clinical Center (CC):
Primary Ciliary Dyskinesia
Variant Cystic Fibrosis
Bronchopulmonary Mycosis
Airway Infection Susceptibility
Bronchiectasis
Chronic Respiratory Infection

Additional relevant MeSH terms:
Autoimmune Diseases
Pancreatic Diseases
Digestive System Diseases
Lung Diseases
Respiratory Tract Diseases
Genetic Diseases, Inborn
Infant, Newborn, Diseases
Immune System Diseases
Bronchial Diseases
Fibrosis
Cystic Fibrosis
Immunologic Deficiency Syndromes
Bronchiectasis
Common Variable Immunodeficiency
Pathologic Processes

ClinicalTrials.gov processed this record on September 18, 2014