Determinants of Daily Physical Activity Level in Patients With Cystic Fibrosis - Pilot Study Protocol...
Cystic FibrosisThe aim of the study is to analyze the determinants (barriers and facilitators) of the daily physical activity level in a population of patients with cystic fibrosis. A population of patients will be included in order to realize an assessment of their daily physical activity level over an entire week. A specific questionnaire, designed from 3 different questionnaires, will be associated to the physical activity evaluation and will allow to asses psycho-social and environmental factors.
Volatile Organic Compounds for the Assessment of Liver Disease
CirrhosisCirrhosis5 moreThis study aims to determine whether a breath test could be used for early detection of hepatic fibrosis, cirrhosis or hepatocellular carcinoma. Patients who are attending for a planned liver outpatient services or investigations will be approached to provide a breath sample. Multi platform mass spectrometry analysis will be performed to establish volatile biomarkers that can discriminate between fibrosis, cirrhosis and hepatocellular carcinoma.
Comparison of Nutritional Screening Tools in Liver Cirrhosis Patients
MalnutritionCirrhosis1 moreThis study compares two nutritional screening questionnaires in cirrhotic patients. All patients will be assessed with both questionnaires, besides a complete nutritional assessment.
Cohort of IPF Patients Experiencing an Exacerbation
Idiopathic Pulmonary FibrosisExtension 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.
Natural History of Bronchiectasis
BronchiectasisCystic Fibrosis2 moreBackground: 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.
Role of Genetics in Idiopathic Pulmonary Fibrosis (IPF)
Idiopathic Pulmonary FibrosisFamilial Pulmonary Fibrosis2 moreThe purpose of this study is to investigate inherited genetic factors that play a role in the development of familial pulmonary fibrosis and to identify a group of genes that predispose individuals to develop pulmonary fibrosis. Finding the genes that cause pulmonary fibrosis is the first step at developing better methods for early diagnosis and improved treatment for pulmonary fibrosis. The overall hypothesis is that inherited genetic factors predispose individuals to develop pulmonary fibrosis.
Chemotherapy and Liver Cirrhosis in Frequently-associated Cancers
CirrhosisNeoplasmsCirrhosis and cancers of the upper digestive tract, colorectal and ENT share common risk factors. Liver cirrhosis can change the elimination of cancer drugs. Precise data on management and outcome of patients with liver cirrhosis undergoing chemotherapy are lacking. Most patients have been excluded from clinical trials evaluating conventional therapies. The study of tolerance, side effects, and outcome in patients with cirrhosis could help improve chemotherapy management for better tolerance and efficacy. The main objective is to estimate the frequency of liver cirrhosis among patients evaluated in CPR for ENT, upper digestive or colorectal cancer. Secondary objective includes the evaluation ofthe impact of cirrhosis on the management of chemotherapy by comparing cirrhotic patients' outcomes with a control group of matched non-cirrhotic patients.
Pulmonary Condensate: Non-invasive Evaluation of Pulmonary Involvement in Asthma and Cystic Fibrosis....
Bronchial AsthmaPulmonary Cystic FibrosisExhaled breath condensate (EBC) represents a rich source for countless biomarkers that can provide valuable information about respiratory as well as systemic diseases. Finding non-invasive methods for early detection of lung injury, inflammation and infectious complications in chronic diseases like (CF) Cystic fibrosis or (AB) Bronchial asthma would be highly beneficial. Investigators propose to establish EBC "breathprints" revealing molecular signatures of pulmonary inflammation and specific respiratory bacterial infections of CF patients and AB. Investigators hypothesize that the analysis of EBC can reveal biomarkers specific for severity of the inflammation, and infection caused by opportunistic pathogens such as P. aeruginosa (PA). With these breath-prints, investigators also propose to establish correlations between respiratory microbiota using traditional methods and CF lung disease severity. Together, the studies will advance the development and validation of EBC as a novel tool for the proper diagnosis of AB and monitoring of CF disease activity, treatment efficacy and PA or another opportunistic infections.
Non-contrast Lung Perfusion Mapping Applied for New Insights in Cystic Fibrosis
Cystic FibrosisCystic fibrosis (CF) results in the thickening of mucus in the lungs and other organs due to dysfunction of a transmembrane conductance protein. This allows buildup of bacteria that results in inflammation, leading to tissue breakdown and loss of function. In the lungs, this process causes loss of air exchange structures progressing to diminished lung function. The exchange of oxygen in the lungs depends on both the integrity of air conduits and vasculature. Most clinical assessments, however, focus on ventilatory function, with the assumption that any vascular compromise is secondary. Nevertheless, there is evidence, some from the investigator's lab, to suggest that perfusion anomalies in the lung occur before signs of ventilatory dysfunction. Thus, the inflammatory processes of CF may impact pulmonary microvasculature specifically and concurrently or prior to damage to ventilatory structures. This study aims to apply a new MRI method to serially measure regional lung perfusion, without the use of contrast agent, in children with CF and to associate it with regional assessments of ventilation and to serum cytokines or proteomic markers of angiogenesis and inflammatory processes. The investigator's lab has recently developed a noninvasive, non-contrast, method of labeling blood flowing into the lungs and generating a map of perfusion. The investigator aims to couple this technique to existing methods using hyperpolarized Xenon to map ventilation. The investigator will apply these methods over time in CF patients, monitoring the relationship between regional perfusion and ventilation defects. This pilot work will provide the foundation for larger studies to establish the essential etiological role of perfusion deficits in CF.
Personalized Long-term Human Albumin Treatment in Patients With Decompensated Cirrhosis and Ascites...
Decompensated Cirrhosis and AscitesALB-TRIAL