Screening for Cystic Fibrosis and Cystic Fibrosis Related Disorders in Chinese Adults With Bronchiectasis

NCT05604495 | Recruiting

• 1 other identifier: 20220922
• Study Type: observational
• Target: 1,000
• Locations: 1 country
• Sites: 1

Brief Summary

The study carries out Sweet Tests and CFTR-mutation screening to explore the prevalence, clinical characteristics, and prognosis of cystic fibrosis, as well as the CFTR-mutation spectrum in Chinese adults with bronchiectasis. The study is multi-centered, prospective, non-interventional, and observational.

Conditions

Cystic Fibrosis, Pulmonary

Outcome Measures

Primary Outcomes (1)

• Sweat chloride and conductivity

  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

  within 14 days after sweat collection

Secondary Outcomes (1)

• CFTR-mutation screening

  1 year

Other Outcomes (6)

• Prevelance of CF or CFTR dysfunction

  1 year

• Microbiology

  through study completion, an average of 1 year

• Bronchiectasis severity Score (BSI)

  At baseline and updated once a year for 3 years

Study Arms (1)

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

Interventions

Sweat Test DIAGNOSTIC_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.

Adult patients with bronchiectasis (unknown cause)

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Patient recruitment relies on the China Bronchiectasis Registry and Research Collaboration. The final study population will be dynamically adjusted according to the incidence of CF in Chinese adults with bronchiectasis, which will be concluded by the primary study conducted on 200 bronchiectasis patients. 1000 bronchiectasis patients are temporarily decided to be enrolled.

You may qualify if:

• Age\>18 years, the diagnosis of bronchiectasis needs a reference to the definition of "non-cystic fibrosis bronchiectasis guideline" published by the British Thoracic Society in 2017 or 2021 China bronchiectasis expert consensus, clinical symptoms of cough and expectoration, with or without intermittent hemoptysis, and chest CT showed bronchiectasis
• Patients who are willing to sign the consent form and participate in the study.

You may not qualify if:

• Patients with incomplete essential information, which is needed for the integrity of data analysis. Essential information includes CT images, respiratory sample cultures, spirometry, and exacerbation history for at least one year.
• Patients under 18 years old.
• Patients with an implanted device, such as a defibrillator, neurostimulator, pacemaker, or ECG monitor.
• Patients with a history of epilepsy or seizures.
• Patients who are pregnant.
• Patients that have a known sensitivity or allergy to any ingredient.
• Over-damaged, denuded skin or other recent scar tissue.
• Patients with Cardiac Conditions or with suspected heart problems.

Sponsors & Collaborators

• Shanghai Pulmonary Hospital, Shanghai, China: lead

Study Sites (1)

Shanghai pulmonary hospital

Shanghai, Shanghai Municipality, 200433, China

RECRUITING

Biospecimen

Retention: SAMPLES WITH DNA

Genomic DNA extracted from peripheral whole blood.

MeSH Terms

Conditions

Cystic Fibrosis

Condition Hierarchy (Ancestors)

Pancreatic Diseases; Digestive System Diseases; Lung Diseases; Respiratory Tract Diseases; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Infant, Newborn, Diseases

Study Officials

• Jin-fu Xu, MD

  Shanghai Pulmonary Hospital, Shanghai, China

  STUDY CHAIR

Central Study Contacts

Jin-fu Xu, MD

CONTACT

+86 13321922898; jfxucn@163.com

Study Design

• Study Type: observational
• Observational Model: CASE ONLY
• Time Perspective: PROSPECTIVE
• Target Duration: 5 Years
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Director, Head of respiratory medicine

Study Record Dates

• First Submitted: October 25, 2022
• First Posted: November 3, 2022
• Study Start: September 7, 2022
• Primary Completion: December 31, 2024
• Study Completion: December 31, 2024
• Last Updated: April 23, 2024

Record last verified: 2024-04

Locations

CN (1)