Role of Body Fat Distribution in Metabolic and Pulmonary Decline in Cystic Fibrosis (ORBIT-CF)
Cystic FibrosisNutrition and body composition, the amount of muscle and fat in the body, has a role in overall health. This study wants to learn more about how nutrition and body composition affects health outcomes like glucose tolerance and lung function in patients with cystic fibrosis (CF) who are ages 16-30 years old. 60 adolescents and young adults with CF will be recruited, and 30 volunteers without cystic fibrosis. A total of 40 of these study participants with CF will be asked to return for annual study visits for 2 years after the first visit. The long-term goal of this study is to use the information collected to make decisions about future nutrition monitoring and interventions which help maintain optimal health for individuals with CF.
Idiopathic Pulmonary Fibrosis and Interstitial Lung Disease Prospective Outcomes Registry
Idiopathic Pulmonary FibrosisInterstitial Lung DiseaseThis registry will collect data on the strategies used to achieve a diagnosis of Idiopathic Pulmonary Fibrosis (IPF) and Chronic Fibrosing Interstitial Lung Disease with Progressive Phenotype (ILD) and the treatment and management efforts applied throughout study follow-up, clinical outcome events and patient reported outcome data. Blood samples will be collected periodically throughout the study for use in future research efforts. For participants with non-IPF, chronic fibrosing ILD with progressive phenotype, HRCT images will be collected throughout the study for use in future research efforts.
BPF Genetics of ILD Study
Lung; DiseaseInterstitial8 moreThe investigators aim to examine the genetic determinants of interstitial lung disease in a cohort of subjects with regular exposure to pigeons, a known cause of one form of interstitial lung disease known as hypersensitivity pneumonitis. In addition we will examine immunological causes for hypersensitivity pneumonitis in this group. We anticipate our work will provide insights of use to clinicians and patients with hypersensitivity pneumonitis and other interstitial lung diseases.
Impact of Sinus Surgery on Individuals With Cystic Fibrosis
Chronic Rhinosinusitis (Diagnosis)Cystic FibrosisThis study will be a prospective, observational study of patients who undergo endoscopic sinus surgery for cystic fibrosis-related chronic rhinosinusitis (CRS). Individuals who do not undergo surgery but are treated medically for CRS will also be enrolled to serve as a control group. Outcomes analyzed will include pulmonary, quality of life, and others.
Evaluation of Lung T1-MRI in Pediatric Cystic Fibrosis Patients
Cystic FibrosisIn this observational study, the investigators evaluate the sensitivity of T1-MRI to identify lung perfusion changes in pediatric patients with CF (age = 6-11) before and after initiating FDA-approved Trikafta therapy. The investigators compare these Lung T1 MRI assessments (% Normal lung perfusion) to currently best-available assessments of lung function in CF patients (i.e., MBW (LCI( and Spirometry (FEV1 % Predicted).
Dyspnea and Idiopathic Pulmonary Fibrosis
Pulmonary FibrosisLongitudinal prospective exploratory study on the evolution of dyspnea, in its sensory and affective dimensions, in patients followed for idiopathic pulmonary fibrosis (IPF), between inclusion and a 6-month evaluation
Assessment of CFTR-Modulator Treatment in Cystic Fibrosis Lung Disease Using Novel Structural and...
Cystic FibrosisIn 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 conventional proton MRI of patients in a hospital.
Comparative and Additive Diagnostic Performance of Magnetic Resonance Elastography (MRE) and Corrected-T1...
Nonalcoholic Fatty LiverNonalcoholic Steatohepatitis2 moreThis pilot study will evaluate conventional and investigational MR imaging and spectroscopic sequences and collect data to help plan more definitive future studies.
Liver Transplantation in Patients With CirrHosis and Severe Acute-on-Chronic Liver Failure: iNdications...
Liver DiseasesLiver Cirrhosis2 moreManagement of ACLF is mainly supportive. The poor outcomes lead physicians to consider liver transplantation as an option, even if controversial. In sicker recipients, LT results in immediate survival, but poor medium-term survival rates in some studies. The scarcity of deceased donors obliges to maximize LT success. Alternative strategies, as living-donor LT, should be explored. LDLT has impressive results in Eastern centers, but it is restrained in Western countries, due to potential life-threatening complications in the donor.
Cirrhosis Registry of Hospitalized Patients
CirrhosisLiver4 moreCirrhosis registry of consecutive adult consenting patients hospitalized with liver cirrhosis in the tertiary liver unit