Cross-sectional Characterization of Idiopathic Bronchiectasis
|First Received Date ICMJE||December 18, 2010|
|Last Updated Date||October 27, 2015|
|Start Date ICMJE||December 2010|
|Primary Completion Date||Not Provided|
|Current Primary Outcome Measures ICMJE||Not Provided|
|Original Primary Outcome Measures ICMJE||Not Provided|
|Change History||Complete list of historical versions of study NCT01264055 on ClinicalTrials.gov Archive Site|
|Current Secondary Outcome Measures ICMJE||Not Provided|
|Original Secondary Outcome Measures ICMJE||Not Provided|
|Current Other Outcome Measures ICMJE||Not Provided|
|Original Other Outcome Measures ICMJE||Not Provided|
|Brief Title ICMJE||Cross-sectional Characterization of Idiopathic Bronchiectasis|
|Official Title ICMJE||Cross-Sectional Characterization of Idiopathic Bronchiectasis|
- Bronchiectasis is a type of lung condition in which the lungs airways are abnormally stretched and widened. This stretching and widening makes it difficult for mucus and other substances to move out of the lungs, encouraging the growth of bacteria and leading to breathing problems or infection. Bronchiectasis can be caused by genetic disorders or diseases such as tuberculosis or rheumatoid arthritis. Researchers are interested in developing better ways to diagnose and treat a lung problem called idiopathic or unexplained bronchiectasis.
- To better describe the physical characteristics, radiographic patterns, and airway microbiology of unexplained bronchiectasis and to look for possible genetic links or risk factors.
The Genetic Disorders of Mucociliary Clearance Consortium (GDMCC) is comprised of 6 geographically-dispersed clinical research sites designed to study chronic disorders of the conducting airways associated with bronchiectasis. The first Clinical Center protocol (06-I-0217) focused on airway diseases with a known genetic etiology: primary ciliary dyskinesia (PCD), variant cystic fibrosis (vCF), and pseudohypoaldosteronism (PHA). This second GDMCC protocol will study adult patients with non-CF, idiopathic bronchiectasis, whose genetic etiologies are not known. Patients will receive the same level of rigorous testing that has allowed proper diagnosis of patients with PCD, vCF, and PHA.
Idiopathic bronchiectasis is reportedly more common in females with certain asthenic morphotypes and associated with environmental organisms, such as nontuberculous mycobacterium (NTM). Gender predilection due to referral bias is unclear. Other susceptibility factors predisposing to bronchiectasis or acquisition of NTM are also unclear.
The study will attempt to broaden the understanding of this disease by comparing gender-associated factors and NTM status. A relatively equal number of both females/males and NTM/non-NTM infected subjects will be accrued. Patients will be stratified by gender and by the presence/absence of respiratory infection with NTM. Approximately 300 people may be screened to find 260 eligible subjects since a small number (e.g., 40 patients) may be diagnosed with PCD, vCF, or other known etiology as an explanation for the bronchiectasis.
This single-visit protocol will use a systematic approach to characterize the clinical, morphological, radiological, and microbiological phenotypes of idiopathic bronchiectasis. Physical features, radiographic patterns, and associated lower airway microbial flora will be assessed. There is no natural history of disease course follow-up component to this protocol. The standard evaluation includes a quantitative assessment of body morphometrics, functional assessments of pulmonary physiology, nasal nitric oxide measurement with subsequent detailed assessment of ciliary structure and motility in selected patients, quantitative immunoglobulins levels to screen for humoral immune defects, and selected genetic analysis of candidate alleles, such as CFTR and A1AT.
Genotype/phenotype correlations will be researched and possibly defined. Careful evaluation and characterization of these phenotypes will guide the genetic characterization of idiopathic bronchiectasis, and likely lead to an improved diagnostic approach. Identification of disease causing genes may provide new therapeutic targets.
|Study Type ICMJE||Observational|
|Study Design ICMJE||Time Perspective: Cross-Sectional|
|Target Follow-Up Duration||Not Provided|
|Sampling Method||Not Provided|
|Study Population||Not Provided|
|Intervention ICMJE||Not Provided|
|Study Group/Cohort (s)||Not Provided|
* Includes publications given by the data provider as well as publications identified by ClinicalTrials.gov Identifier (NCT Number) in Medline.
|Recruitment Status ICMJE||Recruiting|
|Estimated Enrollment ICMJE||270|
|Completion Date||Not Provided|
|Primary Completion Date||Not Provided|
|Eligibility Criteria ICMJE||
The criteria for participants to enter the study mandates that each patient have received a standard (current clinical practice) diagnostic evaluation that includes a CT scan of the chest to document bronchiectasis, prior to enrolling in the Consortium study. To enter this protocol, adults must have bronchiectasis and meet the following criteria:
A participant should not be in the study if they have not had a standard clinical evaluation to rule out other potential causes of chronic sino-pulmonary disease.
|Ages||18 Years and older|
|Accepts Healthy Volunteers||No|
|Listed Location Countries ICMJE||United States, Canada|
|Removed Location Countries|
|NCT Number ICMJE||NCT01264055|
|Other Study ID Numbers ICMJE||110046, 11-H-0046|
|Has Data Monitoring Committee||Not Provided|
|Plan to Share Data||Not Provided|
|IPD Description||Not Provided|
|Responsible Party||National Institutes of Health Clinical Center (CC) ( National Institute of Allergy and Infectious Diseases (NIAID) )|
|Study Sponsor ICMJE||National Institute of Allergy and Infectious Diseases (NIAID)|
|Collaborators ICMJE||National Heart, Lung, and Blood Institute (NHLBI)|
|Information Provided By||National Institutes of Health Clinical Center (CC)|
|Verification Date||July 2015|
ICMJE Data element required by the International Committee of Medical Journal Editors and the World Health Organization ICTRP