Screening for Cystic Fibrosis and Cystic Fibrosis Related Disorders in Chinese Adults With Bronchiectasis
|The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Know the risks and potential benefits of clinical studies and talk to your health care provider before participating. Read our disclaimer for details.|
|ClinicalTrials.gov Identifier: NCT05604495|
Recruitment Status : Recruiting
First Posted : November 3, 2022
Last Update Posted : November 3, 2022
|Condition or disease||Intervention/treatment|
|Cystic Fibrosis, Pulmonary||Diagnostic Test: Sweat Test|
|Study Type :||Observational [Patient Registry]|
|Estimated Enrollment :||1000 participants|
|Target Follow-Up Duration:||5 Years|
|Official Title:||The Clinical and Genetic Characteristics of Cystic Fibrosis and Cystic Fibrosis Disorders in Chinese Adults With Bronchiectasis|
|Actual Study Start Date :||September 7, 2022|
|Estimated Primary Completion Date :||December 31, 2024|
|Estimated Study Completion Date :||December 31, 2024|
Adult patients with bronchiectasis (unknown cause)
Diagnosis of bronchiectasis was performed using chest HRCT scans in suspected patients with coughing and expectoration, or long durations of hemoptysis. High-resolution images were obtained during full inspiration at 1-mm collimation and 10-mm intervals from the apex to the base of the lungs. The presence of bronchiectasis was confirmed based on the following criteria: 1) lack of tapering in the bronchi; 2) dilation of the bronchi where the internal diameter was larger than that of the adjacent pulmonary artery; or 3) visualization of the peripheral bronchi within 1 cm of the costal pleural surface or the adjacent mediastinal pleural surface.
Diagnostic Test: Sweat Test
In people with cystic fibrosis (CF), there is a problem in the transport of chloride across cell membranes. This results in higher concentrations of chloride (as salt) in sweat compared to those who do not have cystic fibrosis. So, if there is a family history or a possibility of CF, the sweat test is part of the special tests to help make, or exclude, a diagnosis of cystic fibrosis.
- Sweat chloride and conductivity [ Time Frame: within 14 days after sweat collection ]Sweat chloride and conductivity is the measured analyte most directly related to the abnormal function of the cystic fibrosis transmembrane regulator (CFTR), the chloride channel that is defective in cystic fibrosis patients
- CFTR-mutation screening [ Time Frame: 1 year ]CFTR-mutation screening will be carried out in Patients with abnormal results of Sweat Test. Mutation detection analysis will be performed in Next Generation Sequencing method using the genomic DNA extracted from peripheral whole blood.
- Prevelance of CF or CFTR dysfunction [ Time Frame: 1 year ]The CF and CFTR dysfunction diagnosis is according to the results of sweat tests combined with CFTR sequencing results. CF is defined as a chloride concentration above 59 mmol/L combined with more than one CFTR mutation, while CFTR dysfunction is defined as a chloride concentration between 30-59mmol/L combined with one or two CFTR mutations.
- Microbiology [ Time Frame: through study completion, an average of 1 year ]Sputum or bronchoalveolar lavage fluid culture
- Bronchiectasis severity Score (BSI) [ Time Frame: At baseline and updated once a year for 3 years ]
The scale is presented online. It consists of 9 items including Age, BMI(Body Mass Index), % FEV1 Predicted, Previous Hospital Admission in the past 2 years, number of exacerbations in previous year, MRC Breathlessness Score, Pseudomonas Colonisation, Colonisation with other organisms,affected lobes.
0-4 Mild Bronchiectasis. 5 - 8 Moderate Bronchiectasis. 9 + Severe Bronchiectasis.
- Lung function (FEV1 %, FVC %, FEV1/FVC %) [ Time Frame: 1 year ]Lung function would be tested by spirometry when patients visit the clinic.
- E-FACED score [ Time Frame: At baseline and updated once a year for 3 years ]It consists of 6 items including exacerbation history in the past year, % FEV1 predicted, Age, Chronic colonization by Pseudomonas aeruginosa, n° of pulmonary lobes affected,and Dyspnea (measured by mMRC score) mild: 0-3 points, moderate: 4-6 points; and severe: 7-9 points
- Comorbidity [ Time Frame: At baseline and updated every year for up to 3 years ]Comorbidity would be recorded.
Biospecimen Retention: Samples With DNA
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): NCT05604495
|Contact: Jin-fu Xu, MD||+86 email@example.com|
|Shanghai pulmonary hospital||Recruiting|
|Shanghai, Shanghai, China, 200433|
|Contact: Jin-fu Xu, MD +8613321922898 firstname.lastname@example.org|
|Study Chair:||Jin-fu Xu, MD||Shanghai Pulmonary Hospital, Shanghai, China|