Active clinical trials for "Fibrosis"

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 allergic diseases, investigators have demonstrated large gene expression gradients between samples taken from the nasal and bronchial airways in different studies. Specifying the cellular variability throughout the respiratory tree in a normal physiological situation is one of the major objectives defined in the establishment of an atlas of all airway cells, as defined in the objectives of the international consortium Human Cell Atlas. The sequencing of the RNAs present specifically in each individual cell ("single-cell RNAseq"), and its comparison with neighbouring cells allows to document the precise cellular contributions, as well as the signalling pathways involved. The development of tissue sampling, stabilization, transport and single cell analysis procedures can be performed on primary respiratory epithelium cultures and can also be extended to respiratory samples from healthy volunteers. This project will analyze gene expression profiles at the single cell level (single cell RNAseq) in volunteers with chronic obstructive pulmonary disease, interstitial pulmonary fibrosis and compared to healthy subjects of the same age. The technical modalities of the samples will be brushing and staged airway biopsies for direct analysis of the samples. This approach will be complemented by an air-liquid interface culture to allow secondary analysis in single cell RNAseq and three-dimensional mapping of the distribution of these cells with single cell in situ analysis. Thanks to sampling at several levels of the respiratory tree (nose, bronchioles, bronchioles), cellular and gene expression variations along the tracheobronchial axis will be exhaustively documented in subjects of different ages, healthy or suffering from pathologies such as chronic obstructive pulmonary disease and interstitial pulmonary fibrosis. These data will serve as worldwide references for comparisons in different physiological and pathological contexts.

Idiopathic Pulmonary Fibrosis(IPF) is the most common idiopathic interstitial lung disease whose cause is unknown. With age and gender, socio-economic factors are the most influential indicators of health. At present there is very little data on socio-economic factors in the IPF. The investigators hypothesize that a lower socio-economic level and / or exposure to various air pollutants may influence the IPF's natural history, including the severity of diagnosis and prognosis of the IPF. The investigators also hypothesize that the deleterious effect of air pollutants is modulated by individual susceptibility (shorter telomeres) and that this effect is related to oxidative stress and shortening of telomeres.

Idiopathic Pulmonary Fibrosis (IPF) is a progressive lung disease marked by reduced exercise capacity and activity-related breathlessness (commonly termed dyspnea). Our previous work has shown that dyspnea during exercise is associated with an increased drive to breathe (inspiratory neural drive; IND). However, little work has been done to understand the mechanisms of exertional dyspnea in patients with mild IPF. The objectives of this study are to compare the acute effects of inhaled nitric oxide to placebo on ventilatory efficiency (VE/VCO2), and IND at rest and during a standard cardiopulmonary exercise test (CPET). Twenty patients with diagnosed IPF with mild (or absent) mechanical restriction and 20 healthy age- and sex-matched controls will be recruited from a database of volunteers and from the Interstitial Lung Disease and Respirology clinics at Hotel Dieu Hospital. Participants with cardiovascular, or any other condition that contributes to dyspnea or abnormal cardiopulmonary responses to exercise will be excluded. After giving written informed consent, all participants will complete 7 visits, conducted 2 to 7 days apart. Visit 1 (screening): medical history, pulmonary function testing and a symptom limited incremental CPET. Visit 2: Standard CT examination conducted at KGH Imaging. Visit 3: assessment of resting chemoreceptor sensitivity, followed by a symptom limited incremental CPET to determine peak work rate (Wmax). Visits 4 & 5 (run-in): familiarization to standardized constant work rate (CWR) CPET to symptom limitation at 75% Wmax. Visits 6 & 7 (Randomized & Blinded): CWR CPET to symptom limitation while breathing a gas mixture with either 1) 40 ppm iNO or 2) placebo [medical grade normoxic gas, 21% oxygen]. The proposed work has the potential to provide important physiological insights into the underlying mechanisms of heightened dyspnea, as well as examine therapeutic avenues to improve quality of life in patients with IPF.

Lung transplant is an option for treating end-stage lung disease in cystic fibrosis (CF). In the United States, more people with CF and low lung function die each year than undergo lung transplant. More than half of people with CF who die without a lung transplant were never referred for consideration. Patient preference not to undergo lung transplant may account for 25-40% of decisions to defer referral. Rates of death without transplant are higher for people with CF who are members of marginalized communities, including those with Black race, Hispanic ethnicity, or low socioeconomic status. Increasing awareness of lung transplant among people with CF, and promoting understanding of the risks and benefits of transplant, can potentially reduce the number of people with CF who die without a lung transplant. The CF Foundation (CFF) lung transplant referral guidelines were developed to optimize the timing of referral for lung transplant. These guidelines recommend annual conversations with people with CF once their forced expiratory volume in one second (FEV1) is <50% predicted. Considering lung transplant as a treatment option before it is medically needed will allow more time to learn about lung transplant and address any barriers to lung transplant that may exist. Investigators are interested in understanding how people with CF use lung transplant educational resources and how one prepares for having discussions and/or making decisions about lung transplant as a treatment option for advanced CF. The purpose of this study is to test whether a research website improves patient preparedness for discussions about lung transplant. Investigators also aim to understand whether there are unique factors that affect people with CF from communities with decreased access to transplant ("communities of concern"). Study involvement will span 6 months and study activities will involve the following: Four Zoom research sessions (15-30 minutes each) Survey assessments Access to a research website that contains educational resources about lung transplant Audio recording of a routine CF clinic visit to determine if and how lung transplant is discussed between a participant and his/her/their CF doctor

This study will assess the safety and tolerability of inhaled LTI-03 in treatment naïve participants with newly diagnosed IPF.

Longitudinal monitoring of inflammation using skin devices may help predict outcomes compared to traditional blood draws

The pandemic forced us to develop home follow-up for patients with chronical diseases . In the same time, the new era of treatment for cystic fibrosis considerably improves the health of patients with this pathology. This study aims to assess the satisfaction of patients and care providers for an alternated follow-up between tele-consults and in-hospital consults during one year for patients with cystic fibrosis treated by Kaftrio® since at least one year and stable.

This Long-term follow-up is designed to evaluate the safety of patient with Alcoholic Liver Cirrhosis who had administered Cellgram-LC in PMC-P-07 study.

The purpose of this prospective study is to analyze function and phenotype of blood neutrophils in cystic fibrosis patients and the impact of Pseudomonas aeruginosa chronic infection, treatment with CFTR modulators and acute exacerbation on blood neutrophils phenotype and function.

The exercise test provides prognostic information about clinical outcomes and quality of life to optimize care for cystic fibrosis patients (pwCF). The exercise test identifies the causes of exercise restriction, adverse exercise reactions, and exercise-related symptoms. The results help to determine and evaluate the impact of exercise programs at PWCF. Peak oxygen uptake (VO2peak) is a prognostic measure of maximum exercise capacity that usually worsens as CF lung disease progresses. The recommended gold standard exercise test at PWCF is a cardiopulmonary exercise test (CPET) performed on a loop ergometer to assess VO2peak and cardiopulmonary responses to exercise. the recommended incremental protocol, consisting of 1-minute phases, should reach VO2peak within 8-12 minutes. Trained operators perform cpets with complex and expensive laboratory equipment, and it is inaccessible and little used by many people internationally. Step tests are low-cost, portable, easily standardized and require minimal space to perform. The 3-Minute Step Test (3MST) is an externally paced test for the assessment of exercise tolerance set at 30 steps/minute for 3 minutes. In adults with CF, 3MST is useful for assessing oxygen desaturation and predicting future increased use of healthcare services. Limitations include the ceiling effect in less severe CF lung disease, and it is very difficult for some with more advanced lung disease. An incremental maximum A-STEP step test has been developed to assess exercise capacity in the CF lung disease December, without floor or ceiling effects, within clinical space constraints and the need for strict infection prevention. A-STEP is a new incremental maximum step test to assess exercise capacity in PWCF without floor or ceiling effects, as an alternative field test to CPET.