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A National Registry on Chinese Patients With Cystic Fibrosis
Cystic fibrosis (CF) is a rare autosomal recessive disease involving multiple organs, especially the lungs and digestive organs. It is most commonly seen in Caucasians. Only a few Chinese CF patients have been described in literature, taking into account the large population of China. The main objectives of this study are to accurately evaluate the prevalence of CF, the status of disease, the diagnosis and treatment, the quality of care, and the health related outcomes in China.
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An Atlas of Airways at a Single Cell Level in Chronic Obstructive Pulmonary Disease, Idiopathic Pulmonary Fibrosis and Controls
The increasing incidence of chronic respiratory disease is a public health problem that affects hundreds of thousands of people worldwide at all ages. Directly exposed to atmospheric airborne contaminants (pollution, allergens), the respiratory tract represents a complex ecosystem involving different cells (multiciliated, basal, mucosecretory, neuroendocrine, etc.) that develop complex interactions with the surrounding connective tissue but also with their rich immune environment and the local microbiota. Although a pathophysiological continuum is postulated between the nasal and bronchial airways in certain diseases, such as...
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An International Patient-led Registry in Fibrotic Interstitial Lung Diseases Using eHealth Technology
The I-FILE study is a prospective multicenter, multinational observational study where the feasibility of a patient-led registry using home monitoring in patients with pulmonary fibrosis will be evaluated. The aim of the study is to gain more insights in disease behavior in patients with pulmonary fibrosis, so in future patients with progressive disease can be better identified.
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Anlotinib Capsules in the Treatment for IPF/PF-ILDs
The use of Anlotinib hydrochloride capsules for the treatment of IPF/PF-ILDs, with FVC as the primary efficacy endpoint to evaluate its effectivenes
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A Non-pharmacological Cough Control Therapy
Coughing affects almost all individuals with ILD leading to physical, psychological and social distress and prevents individuals from performing their activities of daily living, working or socialising in public places. Unfortunately, there are no licensed medications available to treat chronic cough and the few drugs that have been tried resulted in little efficacy and significant side effects. Drug-free cough control interventions have shown promise in reducing the severity and impact of coughing on patients' lives but have not been tested in individuals with ILD. This study aims to explore the feasibility and effectiveness of a...
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A Phase 1 Study to Evaluate Safety, Tolerability, Pharmacokinetics, and Immunogenicity of HuL001
This is a first-in-human, two-part, Phase 1 study that will characterize the safety, tolerability, PK, and immunogenicity of HuL001.
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Assessing the Efficacy of Sirolimus in Patients With COVID-19 Pneumonia for Prevention of Post-COVID Fibrosis
The primary purpose of this study is to determine whether the drug sirolimus reduces the likelihood of developing of pulmonary fibrosis in patients who are hospitalized with COVID-19 pneumonia.
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Assessment of CFTR-Modulator Treatment in Cystic Fibrosis Lung Disease Using Novel Structural and Functional MRI
In this study, MRI of the lungs of healthy volunteers and participants with cystic fibrosis (stable and participants initiating CFTR modulator treatment) will be performed over a period of 6 months to determine if lung MRI is able detect structural and functional abnormalities/changes in early cystic fibrosis disease. During the 6 month period, 3 study visits will occur. 70 subjects aged 6 and older will participate in this study. Xenon MRI is a non-invasive imaging technique that does not involve x-rays or ionizing radiation. Rather, this imaging method utilizes the same hardware and software principles that are used for ...
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Assessment of Continuous Positive Airway Pressure Therapy in IPF
The purpose of this study is to evaluate whether biomarkers of lung injury and remodeling are responsive to effective continuous positive airway pressure (CPAP) treatment in adults with idiopathic pulmonary fibrosis (IPF) and moderate-to-severe obstructive sleep apnea (OSA).
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A Study Evaluating the Efficacy and Safety of Vixarelimab in Participants With Idiopathic Pulmonary Fibrosis and in Participants With Systemic Sclerosis-Associated Interstitial Lung Disease
The main purpose of the study is to evaluate the efficacy of vixarelimab compared with placebo on lung function in participants with idiopathic pulmonary fibrosis (IPF) and in participants with systemic sclerosis-associated interstitial lung disease (SSc-ILD). Participants who complete 52-weeks of treatment in the Double-blind Treatment (DBT) period can choose to enroll in the optional Open-label Extension (OLE) period to receive treatment with vixarelimab for another 52 weeks.
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