Natural History of Bronchiectasis

• PARTICIPANT INCLUSION CRITERIA:

1. Children age 5 years and above and adults referred to the NIH with chronic orrecurring respiratory infections will be eligible for participation.

2. Males and females will be considered without regard to race or ethnicity orupper age limit.

3. Emphasis will be primarily on non CF bronchiectasis for elucidation ofmechanisms of infection susceptibility. However, select patients with cysticfibrosis or acquired immune defects (such as HIV) may be studied if relevanthost defects are suspected, if needed for comparison purposes, or to evaluateand study the management of chronic pathogen associated conditions such asnontuberculous mycobacterial infections or allergic bronchopulmonaryaspergillosis.

4. Subjects must have a primary or referring physician

5. Subjects must be willing to have samples stored

INCLUSION CRITERIA FOR RELATIVES:

As a part of this protocol we may obtain blood, sputum, urine, or buccal swabs from some blood relatives of patients on the study, with the hope of isolating and characterizing the primary host defense defect(s) or genetic links responsible for airway infection susceptibility and/or bronchiectasis seen within families. Male and female relatives will be accepted without limitation due to age. These relatives may have pertinent disease-related history obtained, but will neither receive treatment nor have any other protocol procedures done unless they are enrolled on the study.

PARTICIPANT EXCLUSION CRITERIA:

1. We wish to avoid enrolling subjects, especially children, who have commonrespiratory problems (aeroallergen sensitivity, asthma, gastric esophageal reflux)that are not associated with an underlying abnormality in host defenses. Evidence ofsignificance of chronic or recurring infections suggestive of an underlying airwaysurface or systemic host defense defect should be documented by one or more of thefollowing: a) sinus or lower airway cultures positive for bacterial, fungal, ormycobacterial pathogens characteristic of these defects; or b) radiographic evidenceof sinusitis with mucosal thickening and/or air-fluid levels; or c) radiographicevidence of bronchiectasis; d) severity of otitis media requiring placement oftympanic membrane pressure equalization tubes; e) severity of sinusitis requiringsurgical intervention.

2. Patients who are unable or unwilling to provide informed consent either directly orvia appropriately designated surrogate.

3. Any patient who, in the opinion of the Investigator, is unable or unwilling tocomply with regular follow-up or is unlikely to provide pertinent informationregarding disease progression or response to treatment may be excluded fromlongitudinal follow-up.

PARTICIPATION OF CHILDREN:

Children under the age of 5 will be excluded from this protocol due to the difficulty of performing pertinent assays in infants and younger children, difficulty distinguishing significance of respiratory infections which occur commonly in younger children, and the lack of adequate facilities and equipment for management of children younger than 2 years.