Characterization of Pulmonary Microbiome in Cystic Fibrosis Patients
The composition and role of the pulmonary microbiota is not yet well described in cystic fibrosis patients. The objective of our longitudinal follow-up of primary colonised patients is to show the presence of a link between the composition of the microbiota and the effectiveness of antibiotic therapy. All patients followed at the Montpellier CF center will be asked to participate in this cohort. All patients have a regular follow-up every 1 to 6 months and will be asked at each visit to keep their sputum sample in excess of the analyses requested for their follow-up
Clinical and Basic Investigations Into Hermansky-Pudlak Syndrome
Hermansky-Pudlak Syndrome (HPS) is an inherited disease which results in decreased pigmentation (oculocutaneous albinism), bleeding problems due to a platelet abnormality (platelet storage pool defect), and storage of an abnormal fat-protein compound (lysosomal accumulation of ceroid lipofuscin). The disease can cause poor functioning of the lungs, intestine, kidneys, or heart. The major complication of the disease is pulmonary fibrosis and typically causes death in patients ages 40 - 50 years old. The disorder is common in Puerto Rico, where many of the clinical research studies on the disease have been conducted. Neither the full...
Clinical Course of Interstitial Lung Diseases: European IPF Registry and Biobank
Born out of the European Union 7th Framework Programme funded project European IPF Network (eurIPFnet), the European IPF Registry (eurIPFreg) has become Europe's leading database of longitudinal data from IPF patients, including control groups of patients with other lung diseases. The registry was initiated with the intention of creating a permanent and continuously growing record of well defined data on IPF in Europe, in order to increase the chances of finding better treatment options for this devastating disease. Clinical colleagues who would like to actively participate (both in terms of patient recruitment and data analysis)...
Clinical Efficacy and Safety of Autologous Lung Stem Cell Transplantation in Patients With Idiopathic Pulmonary Fibrosis
Idiopathic pulmonary fibrosis (IPF) is a chronic and ultimately fatal disease characterized by a progressive damage of lung structure and decline in lung function.This study intends to carry out an open, single-center, non-randomized, self control phase I clinical trial. During the treatment, lung stem cells will be isolated from patients' own bronchi and expanded in vitro. Cultured cells will be injected directly into the lesion by fiberoptic bronchoscopy after lavage. After twelve-month observation, the investigators will evaluate the safety and efficacy of the treatment by measuring the key clinical indicators.
Clinical Utility of the 1-minute Sit to Stand Test as a Measure of Submaximal Exercise Tolerance in Patients With Cystic Fibrosis During Acute Pulmonary Exacerbation
The aim of this study is to assess whether the 1 minute Sit to Stand (STS) test can be used as a measure of submaximal endurance during a pulmonary exacerbation of Cystic Fibrosis (CF) for patients in the acute care setting. We hypothesize that if the STS test is a valid measure of submaximal cardiovascular endurance, it will moderately correlate with 6 minute walk distance (6MWD).
Cohort of IPF Patients Experiencing an Exacerbation
Extension of the PFBIO cohort which includes patients with newly diagnosed idiopathic pulmonary fibrosis (IPF) for longitudinal follow-up for up to 5 years. In the PFBIO-EXA extension, patients are included if they experience an exacerbation, or other increase in respiratory symptoms requiring hospital admission, for further collection of clinical and biological data.
Colchicine and Post-COVID-19 Pulmonary Fibrosis
Pulmonary fibrosis is a sequela to adult respiratory distress syndrome (ARDS). 40% of patients with corona virus disease 2019 (COVID-19) develop ARDS, and 20% of them are severe. Clinical, radiographic, and autopsy reports of pulmonary fibrosis were commonplace following SARS and MERS, and current evidence suggests pulmonary fibrosis could complicate infection by SARS-CoV-2 too. Colchicine has a direct anti-inflammatory effect by inhibiting the synthesis of tumor necrosis factor alpha and IL-6, monocyte migration, and the secretion of matrix metalloproteinase-9. It suppress secretion of cytokines and chemokines as well as in vitro ...
Collection of Airway, Blood and/or Urine Specimens From Subjects for Research Studies
The purpose of this study is to obtain biologic materials from the blood, airways and/or urine of normal individuals and individuals with lung disease. The normal are used to establish a set of normal ranges for various parameters. These provide control information when compared to individuals with various pulmonary diseases, and will help in understanding of the etiology and pathogenesis of various lung diseases. The underlying hypothesis is that the pathologic morphological changes in the airway epithelium must be preceded by changes in the gene expression pattern of the airway epithelium and potentially in macrophages.
Combined Effect of CFTR Protein Modulator Drugs and Exercise in Cystic Fibrosis
This study aims to assess the effects of programmed exercise combined with CFTR protein modulator drugs in the cardiorespiratory fitness, strength, functional capacity and agility in a group of young patients with Cystic Fibrosis.
Comparing and Combining Bortezomib and Mycophenolate in SSc Pulmonary Fibrosis
The purpose of this study is to look at whether bortezomib, mycophenolate or the combination of both is better to treat scarring of the lung caused by Systemic Sclerosis.