Official Title
The Clinical and Genetic Characteristics of Cystic Fibrosis and Cystic Fibrosis Disorders in Chinese Adults With Bronchiectasis
Brief Title
Screening for Cystic Fibrosis and Cystic Fibrosis Related Disorders in Chinese Adults With Bronchiectasis
Protocol ID
NCT05604495
Lead Sponsor
Shanghai Pulmonary Hospital, Shanghai, China
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.
Detailed Description
The incidence of cystic fibrosis (CF) or CFTR dysfunction in China remains unclear due to
the absence of a diagnosis and different genetic backgrounds. However, the CF case rate
in western countries is relatively high, up to 1/2500. According to the limited
statistics, the most common classic clinical symptom of Chinese CF patients is
bronchiectasis. Other symptoms often seen in diseased western groups, such as pancreatic
insufficiency, are rarely seen in Chinese patients. Thus, Chinese CF or CFTR dysfunction
patients easily misdiagnose bronchiectasis, Chronic pneumonia, asthma, etc. Sweet test
and CFTR-mutation screening are urgently needed for diagnosing and treating CF or CFTR
dysfunction patients in Chinese adults with bronchiectasis. The study will focus on the
change in CFTR function, genetic susceptibility factors, and disease progression to
explore the incidence, clinical characteristics, and prognosis of cystic fibrosis, as
well as the CFTR mutation spectrum in Chinese adults with bronchiectasis.
Study Period
-
Enrollment Count
1,000 participants
Eligibility Criteria
Inclusion Criteria:
- 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.
Exclusion Criteria:
- 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.
Sweat Test Exclusion Criteria:
- 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.
Filters
Cystic Fibrosis, Pulmonary
RECRUITING
ADULT
OLDER_ADULT