Protocol Number: 09-I-0172

Title:

Natural History of Bronchiectasis

Number:

09-I-0172

Summary:

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.

Sponsoring Institute:

National Institute of Allergy and Infectious Diseases (NIAID)

Recruitment Detail

Type: Participants currently recruited/enrolled

Gender: Male & Female

Referral Letter Required: No

Population Exclusion(s): None

Eligibility 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.

Special Instructions:

Currently Not Provided

Keywords:

Primary Ciliary Dyskinesia

Variant Cystic Fibrosis

Brochopulmonary Mycosis

Airway Infection Susceptibility

Bronchiectasis

Recruitment Keyword(s):

Bronchiectasis

Chronic Respiratory Infection

Condition(s):

Bronchiectasis

Cystic Fibrosis

Autoimmune Disease

Common Variable Immunodeficiency

Investigational Drug(s):

None

Investigational Device(s):

None

Intervention(s):

None

Supporting Site:

National Institute of Allergy and Infectious Diseases

Contact(s):

Patient Recruitment and Public Liaison Office
Building 61
10 Cloister Court
Bethesda, Maryland 20892-4754
Toll Free: 1-800-411-1222
TTY: 301-594-9774 (local),1-866-411-1010 (toll free)
Fax: 301-480-9793

Electronic Mail:prpl@mail.cc.nih.gov

Citation(s):

Barker AF. Bronchiectasis. N Engl J Med. 2002 May 2;346(18):1383-93.Barker AF. Bronchiectasis. N Engl J Med. 2002 May 2;346(18):1383-93.

Morrissey BM, Harper RW. Bronchiectasis: sex and gender considerations. Clin Chest Med. 2004 Jun;25(2):361-72.

O'Donnell AE, Barker AF, Ilowite JS, Fick RB. Treatment of idiopathic bronchiectasis with aerosolized recombinant human DNase I. rhDNase Study Group. Chest. 1998 May;113(5):1329-34.

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