Natural History of Bronchiectasis
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|ClinicalTrials.gov Identifier: NCT00943514|
Recruitment Status : Recruiting
First Posted : July 22, 2009
Last Update Posted : August 12, 2022
- 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.
- To study the natural history of bronchiectasis to identify inherited and immune factors that may explain why certain individuals have chronic recurring infections.
- 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.
- 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.
|Condition or disease|
|Bronchiectasis Cystic Fibrosis Autoimmune Disease Common Variable Immunodeficiency|
|Study Type :||Observational|
|Estimated Enrollment :||900 participants|
|Official Title:||Natural History of Bronchiectasis|
|Actual Study Start Date :||August 1, 2009|
chronic or recurring respiratory infections including pulmonary nontuberculous mycobacterial disease
- Identify causes and/or underlying conditions associated with bronchiectasis [ Time Frame: Patients will be seen at least every 6 months for the first 2 years, and then at 1-5 year intervals to follow the course of their disease for 5 years or until they no longer wish to return. ]Diagnosis of underlying associated conditions such as: a) Variant cystic fibrosis through assessment of sweat chloride, and genetics b) Primary ciliary dyskinesia through assessment of nasal nitric oxide production, genetics, and/or mucosal scrapes for real-time video microscopy of ciliary movement and transmission electron microscopy of ciliary ultrastructure c) Primary immunodeficiency through measurement of quantitative immunoglobulins, specific antigen responses, and lymphocyte phenotyping d) Allergic bronchopulmonary mycosis through measurement of total immunoglobulin E, fungal serologies, and skin test responses e) Autoimmune disease by screening for associated conditions such as inflammatory bowel disease or rheumatoid arthritis f) Alpha-1 antitrypsin deficiency by assessment of protein levels, phenotype, and genetics g) Marfanoid morphotypic features by echocardiography and body anthropometric measurements in adult patients
- Monitor a cohort of bronchiectasis patients to better understand factors associated with progression of disease and to assess outcomes of management and therapeutic strategies. [ Time Frame: Patients will be seen at least every 6 months for the first 2 years, and then at 1-5 year intervals to follow the course of their disease for 5 years or until they no longer wish to return. ]1) Assessment of genetic, systemic immune, and/or epithelial surface defense mechanisms involved in airway infection susceptibility and/or in the development of bronchiectasis through the: a) Collection and storage of blood and sputum b) Use and storage of other relevant biologic specimens, such as bronchoalveolar lavage fluid or respiratory epithelium collected under separate protocols or for clinical purposes 2) Assessment of clinical outcomes utilizing standardized measures during follow-up visits to include: a) St. George s Respiratory Questionnaire b) Pulmonary Symptom Severity Score c) Medical Research Council Dyspnea Scale d) Six-minute walk assessment: distance in meters, minimum oxygen saturation, maximum heart rate e) Computerized tomography score f) Spirometry: percent of predicted normal for forced expiratory volume in one second (FEV1%) and forced vital capacity (FVC%) g) Lower airway microbiology
- Define mechanisms and pathophysiology for the development and progression of bronchiectasis [ Time Frame: Patients will be seen at least every 6 months for the first 2 years, and then at 1-5 year intervals to follow the course of their disease for 5 years or until they no longer wish to return. ]Genetic, systemic immune, and/or epithelial surface defense mechanisms involved in airway infection susceptibility and/or development of bronchiectasis through the
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): NCT00943514
|Contact: Chevalia Robinson, R.N.||(301) firstname.lastname@example.org|
|United States, Maryland|
|National Institutes of Health Clinical Center||Recruiting|
|Bethesda, Maryland, United States, 20892|
|Contact: For more information at the NIH Clinical Center contact Office of Patient Recruitment (OPR) 800-411-1222 ext TTY dial 711 email@example.com|
|Principal Investigator:||Kenneth N Olivier, M.D.||National Heart, Lung, and Blood Institute (NHLBI)|