Clinical Research Directory

Natural History of Bronchiectasis

Background: * 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. Objectives: \- To study the natural history of bronchiectasis to identify inherited and immune factors that may explain why certain individuals have chronic recurring infections. Eligibility: * 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. Design: * 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.

The AIRTIVITY™ Study: A Study to Find Out Whether BI 1291583 Helps People With Bronchiectasis

This study is open to adults and adolescents aged 12 to under 18 with bronchiectasis. People can participate in this study if they produce sputum and have had flare-ups (also called exacerbations). The purpose of this study is to find out whether a medicine called BI 1291583 helps people with bronchiectasis. Participants are put into 2 groups randomly, which means by chance. One group takes BI 1291583 tablets and the other group takes placebo tablets. A placebo tablet looks like the BI 1291583 tablet but does not contain any medicine. Participants take 1 tablet once a day for up to 1 year and 6 months. Participants are in the study for up to 1 year and 8 months. During this time, participants visit the study site up to 10 times and get about 13 phone calls from the site staff. Participants regularly complete a diary on a smartphone about their bronchiectasis symptoms and study doctors regularly check for any changes. The study doctors document when participants experience flare-ups. The number of flare-ups is compared between the participants who receive BI 1291583 and those who receive the placebo. The study doctors also regularly check participants' health and take note of any unwanted effects.

Clairleafᵀᴹ: A Study to Test Long-term Treatment With BI 1291583 in People With Bronchiectasis Who Took Part in a Previous Study With This Medicine

This study is open to adults aged 18 years and older with bronchiectasis. People can join the study if they were previously enrolled in another study with BI 1291583 (1397-0012: Airleafᵀᴹ or 1397-0013 Clairaflyᵀᴹ). The purpose of this study is to find out whether a medicine called BI 1291583 helps people with bronchiectasis, an inflammatory lung condition. The investigators also want to know how well people with this condition can tolerate BI 1291583 in the long term. Participants take a low, medium, or high dose of BI 1291583 as a tablet once a day for up to 1 year. Participants who were taking placebo in the AirleafTM or ClairaflyTM study are put into the BI 1291583 dosage groups randomly, which means by chance. Placebo tablets look like BI1291583 but do not contain any medicine. Participants who were taking BI 1291583 in the AirleafTM or ClairaflyTM study continue to take the same dose. Participants visit the study site 10 times and get 4 phone calls from the site staff. During the visits, the doctors collect information on any health problems of the participants. The doctors also check whether BI 1291583 helps reduce the symptoms of bronchiectasis.

Immune Status and Disease Control of Inflammatory Airway Diseases

The goal of this study is to learn how the body's immune system affects disease control in people with different airway inflammatory diseases.We want to understand: 1.Whether specific immune cell patterns in the blood are linked to how severe the disease is or how well it is controlled. Participants will: 1. Answer questions about their health and symptoms. 2. Give blood samples 3. Have lung function tests and other standard check-ups. 4. share sleep study results. We will compare people with airway diseases to healthy volunteers to see how their immune systems differ.

Clinical Outcomes of BronchiEctaSis in Taiwan: A Multicenter Prospective Cohort (BEST Study)

Bronchiectasis is a disease characteristic by bronchial dilatation. Bronchiectasis leads to further impairment of mucociliary clearance, causing a vicious cycle of further infection and progressive airway damage, resulting in poorer control of the primary disease. The clinical presentation is non-specific. The etiologies of bronchiectasis include post-infection, such as bacterial pneumonia, tuberculosis, or aspiration pneumonia, autoimmune disease, such as rheumatoid arthritis, or sjogren's syndrome, cystic fibrosis and immunoglobulin deficiency. The comorbidities of bronchiectasis include cardiovascular disease, cancer such as lung cancer, or esophageal cancer, gastro-oesophageal reflux disease and iron deficiency anemia. Treating these comorbidities will help for the prognosis of bronchiectasis. The treatment for bronchiectasis included airway clearance therapies, mucoactive medication, long term antibiotics and bronchodilator if indicated. Long term anti-inflammatory therapies are not suggested. Surgery is indicated if medical treatment failure and localized bronchiectasis or massive hemoptysis. The different characteristics of bronchiectasis have been reported in different foreign studies. There were few studies in Taiwan. This is a prospective study to find the characteristics including comorbidities, severity, treatment in stable and exacerbation condition and pulmonary rehabilitation in Taiwan.

Expand Pulmonary Rehabilitation to Other Chronic Respiratory Diseases Than COPD

The purpose of this pilot randomized clinical trial is to evaluate the willingness, acceptance, adherence and signs of benefits of structured home-based pulmonary rehabilitation (HPR) and supervised pulmonary tele-rehabilitation (PTR) on respiratory symptoms, quality of life, functioning and physical activity in patients with ILD (Interstitial lung disease, Idiopathic pulmonary fibrosis, Sarcoidosis), Bronchiectasis and Asthma who have a clinically assessed need for a pulmonary rehabilitation program. The main questions the project aims to answer are: * Will HPR and PTR appeal to a minimum of 30% of the eligble patients * Be greatly accepted (≥70% of participant complete 70% or more of the planned sessions) by patients agreeing to participate in the study * Will PTR and HPR lead to positive changes in respiratory symptom relief, symptom relief in general and improve or maintain physical functioning larger than usual care Researchers will compare with a comparable control group who gets usual care (scheduled controls and medication but no exercise intervention) to examine the willingness to be randomized and to get an indication on possible health related effects. Participants will be randomly assigned to one of three groups: 1. A structured supervised group-based PTR (exercise twice a week; each session consists of 35 min. evidence-based exercises and 25 min. of patient education) 2. An individual HPR program (self-initiated physical activity) with motivational and professional counseling once a week (10-15min), supported by a tablet screen 3. A control group receiving usual care (CON)

A Study to Investigate the Safety, Tolerability, and Pharmacokinetics of Inhaled CHF6333 After Single Doses in Healthy Volunteers and After Single and Repeated Doses in Subjects With Bronchiectasis

The aim of this clinical trial is to assess the safety of: * single doses of the study drug CHF6333 in Healthy Volunteers (HVs) and in subjects with Bronchiectasis (BE) - Part I * repeated doses of the study drug CHF6333 in subjects with BE - Part II

Simeox 200 US Study

The purpose of this single arm post-market observational study is to evaluate the short-term effectiveness of the Simeox 200 airway clearance device in improving airway clearance in adult patients aged 18 to 85 with bronchiectasis and chronic mucus hypersecretion. The study aims to establish a baseline for wet sputum weight production using the Simeox 200 device. Additionally, the study aims to assess the short-term effects of Simeox 200 on various outcomes, including lung function, respiratory symptoms, and health-related quality of life. The study participants will undergo the following steps: 1. Baseline Visit (V0): Participants will attend a baseline visit for eligibility screening, consent, a physical exam, and baseline assessments of various relevant parameters such as lung function, SpO2, and Patient-Reported-Outcomes. 2. Treatment Sessions (V1): Participants will perform two supervised treatment sessions with the Simeox 200 device at the clinic, during which sputum will be collected and weighed. 3. Home Use : Participants will use the device at home twice daily for 2 weeks, recording their usage and symptoms in a daily electronic diary. 4. Final Clinic Visit (V2): Participants will return for a final clinic visit for repeat assessments. 5. Follow-up Phone Call (14 days after V2): Participants will receive a follow-up phone call to assess ongoing symptoms and health status.

Evaluation Of The Lung Microbiome In NTM Bronchiectasis

A biomarker cohort study design is proposed to study whether specific airway microbiota alterations are associated with pulmonary Non-tuberculous mycobacteria (NTM) disease. In a cohort of 200 subjects suspected of having pulmonary NTM disease, the investigators will evaluate the airway microbiome using an aliquot of the induced sputum and upper airway samples. Since induced sputum may reflect different regions of the upper/lower airways, the investigators will evaluate the upper and lower airway microbiome in a subgroup (case-control group) of patients using samples obtained through upper airway sampling and bronchoscopy, respectively.

A Cohort for Inflammatory Respiratory Diseases: From Phenotyping to Personalised Medicine

Chronic inflammatory pulmonary diseases, including asthma, chronic obstructive pulmonary disease (COPD), bronchiectasis, cystic fibrosis (CF), primary ciliary dyskinesia (PCD) and interstitial lung diseases (ILD) are characterised by lung inflammation and remodelling. Clinical, functional, microbiological, biological, pathological and prognostic features are highly variable and heterogeneous. Several phenotypes have been described within the same pathology, as similar phenotypic traits between different pathologies, or the coexistence of components of several diagnoses in the same patient, suggesting shared underlying mechanisms that could represent new therapeutic targets, beyond the initial medical diagnosis. The objectives of this prospective study are to analyze the phenotypic characteristics (clinical, demographic, biological, morphological, pathological, and microbiological characteristics) together with respiratory exposures and underlying mechanisms involving airway epithelium and inflammation processes in a cohort of patients diagnosed with asthma, COPD, bronchiectasis, CF, PCD and ILD.

Functional Inspiratory Training in Bronchiectasis

Bronchiectasis is a chronic respiratory disease clinically characterized by recurrent pulmonary exacerbations, chronic cough, and sputum production, and is associated with an increase in the airway-artery ratio and permanent airway dilatation. It is recognized as the third most common chronic respiratory disease after chronic obstructive pulmonary disease (COPD) and asthma. In the International Classification of Diseases and Related Health Problems (ICD-10), it has its own diagnostic code (J47.9). In bronchiectasis, mucociliary clearance is impaired due to bronchial dilatation, and the insufficient clearance of bacteria and mucus from the respiratory tract leads to persistent infection, inflammation, and further airway damage. Progressive airway damage results in impaired lung function, worsening of symptoms, and, ultimately, respiratory failure and death. It can represent the final pathway of various infectious, allergic, inflammatory, genetic, and degenerative disorders, making it one of the most complex and heterogeneous syndromes. Some patients present with daily symptoms, while others remain asymptomatic except during exacerbations. The most common symptom is chronic cough caused by purulent or mucopurulent sputum. Other symptoms include dyspnea, pleuritic chest pain, wheezing, fatigue, and weight loss. Fever, however, is not a common finding and differs from pneumonia. The most frequently used classification system categorizes bronchiectasis into varicose, cylindrical, and saccular (cystic) types. In the most severe form, the saccular type, the bronchi lose their structural integrity completely, forming cystic structures filled with secretions. The mildest form is cylindrical, in which the bronchi have thick, straight walls. In the varicose type, localized narrowings are observed. Diagnosis is made following history and physical examination, with high-resolution computed tomography (HRCT) being the most sensitive and specific method, along with sputum culture and chest radiography. The goals of bronchiectasis treatment are to manage symptoms, prevent complications, and improve quality of life. Both pharmacological and patient-managed interventions are commonly applied. Pharmacological treatments include antibiotics, bronchodilators, and corticosteroids, which are generally administered via inhalation. Patient-managed interventions require active participation and behavioral modification. Within published guidelines, airway clearance techniques are widely described under pulmonary rehabilitation (PR), but there is no consensus regarding PR itself. For individuals with reduced exercise tolerance, participation in PR and engagement in physical activity are recommended. However, research has shown that referral rates to PR are often low, and exercise and physical activity are not consistently prescribed. Within PR, techniques such as the active cycle of breathing techniques (ACBT), postural drainage, thoracic expansion exercises, and various airway clearance methods are applied, with inspiratory muscle training (IMT) forming an important component. Functional inspiratory muscle training (FIMT) is an IMT program developed by considering not only the ventilatory roles of the respiratory muscles but also their non-respiratory functions. FIMT integrates inspiratory muscle training with core stabilization and postural control exercises. The rhythmic co-contractions of the muscles in the core region support trunk stability and provide the basis for movement. In the first stage of the program, participants receive IMT, and subsequently, core stabilization training and dynamic trunk activation exercises are incorporated into the intervention. A review of the literature reveals that no studies have investigated the effectiveness of FIMT in adults with bronchiectasis. In our study, we aim to evaluate the effects of FIMT, applied in addition to home-based chest physiotherapy, on pulmonary function, diaphragm thickness, balance, core stability, functional capacity, physical activity, posture, and quality of life in adults with bronchiectasis. Furthermore, we aim to compare these outcomes with those of IMT applied alongside home-based chest physiotherapy. We believe that this approach will contribute to the development of treatment strategies in clinical practice and help address existing gaps in the literature.

Itraconazole Therapy In Bronchiectasis With Airway Mold

The primary objective of this study is to evaluate the feasibility of itraconazole therapy in patients with bronchiectasis and airway mold. Feasibility will be assessed through recruitment success, treatment adherence, tolerability, and participant retention. The study will also explore the impact on respiratory symptoms and airway microbiome diversity.

Evaluation of the Quality of Life of Patients With DBB With the Installation of OPEP

To carry out an assessment of compliance and quality of life in a population (children and adults) with DDB before and after the use of an oscillating PEP. The aim is to determine the impact of the use of oscillating PEP on the daily life of this population in particular.

Development of an Interactive Digital Respiratory Game and Device and Investigation of Its Effectiveness in Children With Bronchiectasis

Our study aims to develop a personalized interactive digital breathing game and device that can be produced in our country and to provide daily airway cleaning treatment at home via mobile phone or computer without the need for a physiotherapist or healthcare professional with the use of an interactive digital breathing game.

Research on the Mechanism Affecting Progression of Bronchiectasis

Bronchiectasis is a chronic inflammatory respiratory disease defined as the irreversible dilatation of one or more bronchi and is associated with chronic and frequently purulent expectoration, multiple exacerbations and progressive dyspnea. Bronchiectasis has a large heterogeneity. Different patients with bronchiectasis may have different etiology, clinical manifestations, and imaging features. Previous studies showed that there are significant relationship between the airway microbiome and the severity of the disease. For example, patient with airway Pseudomonas aeruginosa colonization has heavier symptoms, heavier severity, poorer quality of life, more acute exacerbations, and worse prognosis. A large number of studies have reported that long-term treatment of low-dose macrolides such as azithromycin or clarithromycin has anti-inflammatory and immunomodulatory effects, which can improve the clinical symptoms and disease progression of various chronic airway diseases, such as diffuse panbronchiolitis, chronic obstructive pulmonary disease, bronchiectasis. Both the 2017 European Respiratory Society guidelines and the 2019 British Thoracic Society Guideline recommend macrolide drugs for the treatment of chronic Pseudomonas aeruginosa colonization bronchiectasis or frequent acute exacerbations bronchiectasis, but the specific mechanism is unknown.This study is based on omics methods (Microbiology and Metabolomics) to deeply explore the composition of airway and gut microbiota in patients with bronchiectasis, the factors affecting the colonization of Pseudomonas aeruginosa and the mechanism of macrolides in the treatment of bronchiectasis. This project is a multicenter clinical study involving patients with bronchiectasis from Wuhan Union Hospital, Guizhou Provincial People's Hospital, and Yichang Central People's Hospital. Patients with bronchiectasis were recruited according to the inclusion and exclusion criteria. Clinical data were collected from these patients (including demographic information, clinical characteristics, pulmonary function, and lung imaging), along with spontaneously expectorated sputum, feces, and peripheral blood, and the patients were followed for 24 months. The microbiome, metabolome, and cytokines in sputum and feces were assessed, as well as cytokines, inflammatory mediators, and metabolites in peripheral blood. Through the above methods,investigators further understand the mechanism affecting progression of bronchiectasis and some factors that lead to the colonization of Pseudomonas aeruginosa, as well as mechanisms of macrolides in the treatment of bronchiectasis.

Effects of Environmental Fungal Exposure on Bronchial Asthma, ABPA and Bronchiectasis

This study is a prospective, observational study of patients aged 18-80 years with clinical diagnosis of bronchial asthma, allergic bronchopulmonary aspergillosis (ABPA), bronchiectasis and healthy subjects. Among them, the bronchial asthma group will be divided into asthma control group, asthma partial control group and asthma uncontrolled group according to the GINA asthma control classification. Record the baseline information of the subjects in detail, including basic information, disease course, smoking, previous acute attacks and hospitalizations, long-term medication, etc. (including the frequency, dose and duration of ICS use, antifungal drug type, dose, duration of use, and course of treatment), and evaluate and record environmental factors (such as indoor).Humidity, temperature, ventilation, pet keeping, plant planting, etc.) and other lifestyle factors that may affect disease control and fungal exposure. The patient's disease status was assessed using questionnaire scores. Sputum samples were taken at the time of enrollment.Set (asthma ABPA group uses sputum induction), pulmonary function test and bronchodilator test, FeNO measurement (asthma ABPA group only), blood routine test, Aspergillus-specific IgE and IgG detection, total immunoglobulin IgE, allergen detection, etc. Dust was collected indoors (bedrooms), outdoor (balconies) and on the surface of air conditioners or fans (if applicable) in the subject's living environment, and environmental data such as ambient temperature, humidity, and particulate matter concentration were recorded. 18S rRNA technology was used for sputum and dust fungus detection, and ELISA was used for asthmatitis symptomatic markers, which assess the impact of fungal infections on the disease. Follow-up for each subject 6 months, 6 months after enrollment, the patient's symptom changes, acute exacerbations/exacerbations, and prognosis were recorded, and relevant questionnaire scores were completed.

Study on the Correlation Between Metabolomics and Anxiety and Depression in Bronchiectasis

Bronchiectasis is a common lung disease. Approximately 20-40% of patients with bronchiectasis experience comorbid anxiety and depression. Multiple studies have now demonstrated that anxiety and depression are associated with an increased risk of disease exacerbation in these individuals. Therefore, this study aims to collect data on anxiety and depression status, disease exacerbation frequency, hospitalisation rates, and mortality among participants diagnosed with bronchiectasis. Concurrently, biological samples including blood, sputum, and stool will be obtained. Through metabolomics analysis, we will investigate the expression of anxiety and depression-related metabolic pathways and identify corresponding biomarkers to explore their role in the progression of bronchiectasis.

Microbial Colonization Distribution and Adaptive Evolution of Lower Respiratory Tract in Bronchiectasis Patients.

Qualified sputum samples from bronchiectasis patients were collected regularly every month for three consecutive years to analyze the microbiome changes of lower respiratory tract of bronchiectasis patients by metagenomic sequencing. Pseudomonas aeruginosa was isolated and the whole genome was sequenced to analyze the adaptive evolution，including virulence, quorum sensing and drug resistance under host pressure. The aim of the study is to clarify the rule of microflora colonization distribution and adaptive evolution in the lower respiratory tract of patients with bronchiectasis, to predict the acute attack and prognosis of patients with microbiome changes, and to find more new prevention and treatment methods by adjusting the microbiome of the lower respiratory tract.

Acoustic Waveform Respiratory Evaluation

The study will evaluate the feasibility of using smartphone speakers and microphones to evaluate the caliber of the airways, detect airway obstruction, aid in airway disease diagnosis, and identify disease exacerbations.

Sleep Quality in Patients With Non-cystic Fibrosis Bronchiectasis

Non-cystic fibrosis (non-CF) bronchiectasis is a chronic, heterogeneous airway disease characterized by irreversible bronchial dilatation, persistent airway infection, and neutrophilic inflammation that together drive daily cough, sputum production, recurrent exacerbations, and progressive functional decline. Sleep is a key determinant of Health-Related Quality of Life (HRQoL) and cardiometabolic health. In chronic lung diseases, nocturnal hypoxemia, cough, dyspnea, and systemic inflammation commonly disrupt sleep continuity and architecture. In bronchiectasis specifically, several studies show high rates of poor sleep quality using the Pittsburgh Sleep Quality Index (PSQI), with \~50-60% of clinically stable patients classified as "poor sleepers," often accompanied by daytime dysfunction. Poor sleep correlates with disease severity indices and symptoms such as nocturnal cough.

A Study to Assess the Effectiveness and Safety of GSK3862995B in Adults With Bronchiectasis

This study is designed to find out how effective and safe a new drug, GSK3862995B, is for adult participants with bronchiectasis, a chronic lung disease. The study will also test how the body processes the drug and to check for any immune reactions. Participants will be divided into groups randomly to receive either one of two different doses of the study drug or a placebo. The main goal of the study is to see how well the drug works compared to the placebo in helping those with bronchiectasis.

OLE Therapy With BE Patients in Home Care Study

The study will examine how the performance of Oscillating Lung Expansion (OLE) therapy affects the respiratory health of patients with Bronchiectasis (BE). The patient will use the BiWaze Clear system for their airway clearance therapy, instead of their previously prescribed therapy. BiWaze Clear is an FDA-cleared respiratory therapy for assisting patients in loosening and mobilizing secretions as well as treating and preventing atelectasis by providing lung expansion and high-frequency oscillation therapies, combined with aerosol delivery. The patient will perform airway clearance with BiWaze Clear as prescribed. The system uses a disposable breathing circuit, which is a single patient use, disposable circuit. The treatment duration is 6 months . The BiWaze Clear System is indicated for the mobilization of secretions, lung expansion therapy, the treatment and prevention of pulmonary atelectasis and has the ability to provide supplemental oxygen when used with an oxygen supply.

Health Impact of Non-Tuberculous Mycobacteria Pulmonary Disease (NTM-PD)

Nontuberculous mycobacteria (NTM) are environmental organisms found in soil and water. The majority do not cause human disease. When they do, this is mostly as a chronic lung infection in people with long-term lung problems such as chronic obstructive pulmonary disease (COPD), bronchiectasis, or cystic fibrosis. The number of people with NTM pulmonary disease (PD) is increasing, and its management can be complex, requiring prolonged treatment with multiple, often toxic, drugs in someone who may already be frail. Non-drug approaches, such as airway clearance techniques, structured exercise, nutritional support and psychological care are used to help manage bronchiectasis and COPD. However, there is limited evidence about their benefit in people with NTM-PD. Also, it is not clear whether these patients' health needs are different from people with bronchiectasis alone. The investigators want to identify the most important symptoms encountered by people with NTM-PD and patient preferences for care. The study also aims to explore whether the need for non-drug measures differs between people with and without NTM-PD who have other underlying lung disease. The research will take place at one NHS centre and involve a single assessment of 40 people with NTM-PD not using specific antibiotics to treat their NTM and 40 people with bronchiectasis but no evidence for NTM. Following consent, and mainly using questionnaires, participants will be asked about their physical and mental health, and nutritional status. Exercise capacity, muscle strength and body muscle/fat composition will also be assessed using simple tests. The total time required will be a maximum of one hour. Recruitment to the study will last around six months. The results will help improve understanding of specific needs of people with NTM-PD and guide clinically relevant research in this area.

Study on Disease Progression and Nutritional Status in Bronchiectasis

Bronchiectasis is a common lung disease. The Bronchiectasis Severity Index (BSI) is a widely used assessment system. The body mass index (BMI) is a commonly used measure of nutritional status, but it has its limitations. To provide a more comprehensive assessment, the investigators also consider other nutrition-related indices such as upper arm circumference, calf circumference, skinfold thickness and grip strength. The investigators will specify the relationship between nutritional status and disease progression by measuring nutrition-related indicators and tracking participants' disease progression.