
Prevalence and Predictive Factors of Portal Vein Thrombosis in Patients With Cirrhosis
Liver CirrhosisPortal Vein ThrombosisSeveral studies have confirmed that patients with cirrhosis possess an imbalance in procoagulant versus anticoagulant activity due to increased factor VIII and decreased protein C. Moreover, in the last two decades there has been an increased recognition that not only bleeding but also thrombosis complicates the clinical course of cirrhosis. The prevalence and pathogenesis of portal vein thrombosis (PVT) in patients with cirrhosis without hepatocellular carcinoma are not clearly defined. The Aim of this study is to assess the prevalence of portal vein thrombosis in patients with cirrhosis without hepatocellular carcinoma, and to prospectively assess the risk factors, outcome, and prognosis in these patients. The investigators plan to enroll two hundred patients with liver cirrhosis. The patients are going to follow up for one year and evaluate at baseline and every 6 months by liver function tests, coagulation test, upper abdomen ultrasound. All relevant clinical events will be evaluated at every follow up.

MR Imaging of Diffuse Myocardial Fibrosis in Transfusion-Dependent Anemias
Thalassemia MajorChronic Blood Transfusion Related Iron OverloadCardiac failure is the major cause of death in patients with thalassemia and chronic blood transfusion-related iron overload. The treatment of thalassemia has been revolutionized over the past decade with the implementation of cardiac MRI based assessment of iron overload. This has enabled detection of cardiac iron overload prior to symptomatic heart failure and now allows for timely therapy which has resulted in a substantial decrease in mortality. However, currently implemented MR imaging techniques assess for iron content only and not for iron related diffuse fibrosis which play a role in iron related heart failure. Histopathologic studies indicate that patients with iron overload have diffuse interstitial fibrosis. Quantitative MR techniques have shown that patients with various cardiomyopathies demonstrate diffuse myocardial fibrosis and that these changes correlate with changes in cardiac function. The investigators propose that quantitative cardiac MRI for assessment of diffuse myocardial fibrosis can further improve our ability to detect early damage to the myocardium and prevent morbidity and mortality from cardiac iron overload. Detection of fibrosis in patients with thalassemia may allow for earlier identification of cardiomyopathy when compared to other techniques in clinical use including T2* analysis. Identification of fibrosis could affect patient management as it would allow for tailoring of iron chelation therapy and may lead to better understanding of the disease processes contributing to heart failure and arrhythmia in these patients.

Expanded Access Program of Nintedanib in Patients With Idiopathic Pulmonary Fibrosis
Idiopathic Pulmonary FibrosisTo provide early access and to evaluate the safety and tolerability of nintedanib in patients with idiopathic pulmonary fibrosis (IPF)

Effect of Beta-adrenergic Blockers on Cardiac Function, Systemic and Splanchnic Haemodynamic and...
Hepatic CirrhosisMulticentric, observational and prospective study with two groups of treatment: Refractory ascites and non-refractory ascites. All patients should be prescribed beta-adrenergic blockers as primary or secondary profilaxis for variceal bleeding.

Evaluation of Liver Fibrosis in HIV-infected Patients With Metabolic Syndrome
Abdominal Obesity Metabolic SyndromeHIV1 moreThis study aims to estimate the prevalence of bridging liver fibrosis and cirrhosis (METAVIR score ≥ F2) according to METAVIR score in HIV infected patients not chronically infected by viral hepatitis but exhibiting a metabolic syndrome according to the IDF definition (International Diabetes Foundation).

Evaluating the Impact of Chronic Rhinosinusitis on the Health-Related Quality of Life Among Adults...
Cystic FibrosisIndividuals with Cystic Fibrosis (CF) have a defective protein, which is known as the cystic-fibrosis transmembrane regulator (CFTR). The CFTR transports salt and hydrates mucous. CFTR defects may result in the accumulation of thick mucous in the sinus cavities. As a result, the tiny hair-like structures that sweep mucous out of the sinuses cannot function properly, which can lead to recurrent infection and swelling of the sinus walls. When symptoms are persistent for more than 12 weeks, this is known as chronic rhinosinusitis (CRS). The symptoms that are associated with CRS are nasal discharge, congestion, facial pain or pressure and reduced sense of smell. CRS in non-CF patients affects a large number of individuals in Canada and has been found to be associated with poor quality of life. In the CF population the life expectancy is increasing but chronic disease like CRS is becoming increasingly prevalent. Investigators currently do not know the impact that CRS has on the health-related quality of life in adults with CF and how many suffer from symptoms. The investigators aim to determine the impact of CRS among adults with CF, in order to gain a better understanding of chronic disease among these individuals. The investigators strongly feel this research will improve the referral processes between Respirologists and Otolaryngologists, thereby improving treatment and quality of life for patients.

IL-17 Neutrophils in CF Lung Inflammation
Cystic FibrosisThe purpose of this study is to determine whether IL-17 polymorphonuclear leukocytes (PMNs) are central to the disease pathology in CF. This will be determined by demonstrating that IL-17 PMNs are present in the CF airway, correlate with lung function measures, and decrease in patients being treated with IV antibiotics for a pulmonary exacerbation.

Phase Angle and Mortality in Patients With Cirrhosis
MalnutritionA few PhA cut-off points have been proposed to define malnutrition in cirrhosis, and they have proven to be useful in prognosticating severity of the disease and mortality. However, PhA needs specific validation in different ethnic groups. The aim of this study was to evaluate the PhA cut-off value that would best define malnutrition and predict mortality in patients with liver cirrhosis.

A Novel Assay to Quantify Treatment Response in Cystic Fibrosis (CF)
Cystic FibrosisInflammation is present in the Cystic fibrosis (CF) airway from the time of infancy, and worsens with the onset of chronic infection. Therapies with proven benefit are associated with decreased airway inflammation. Thus, sensitive and reproducible biomarkers of airway inflammation have long been sought as a necessary component to improved clinical care and to facilitate therapeutic trials for CF. FEV1, the standard outcome measure in CF, is recognized as an insensitive outcome measure. the investigators have identified a panel of 10 genes which sensitively predict resolution of pulmonary inflammation, in response to therapy of an acute pulmonary exacerbation. With the goal of yielding a technically simple but unique CF biomarker assay, the investigators have tested whether proteins signified by these genes show large changes in expression following treatment of acute pulmonary exacerbations. Protein quantifications are among the most common measurements performed in clinical laboratories around the world. Based on preliminary findings that changes in white blood cell proteins mirror changes seen in the genes, the investigators propose to identify top candidate proteins, from the investigators gene panel, which change in response to exacerbation therapy. Once identified, these proteins will be quantified directly with a new blood test which is inexpensive and simple to perform. The investigators propose to validate this blood test in a single site trial. If successful, this proposal will yield a biomarker assay that will be available to validate in a multi-site trial and provide unique insights into mechanisms that regulate white blood cell activation and recruitment in CF lung disease.

Prospective Validation of Cough, Dyspnea, and Quality of Life Questionnaires in Patients With IPF...
Interstitial Lung DiseaseIdiopathic Pulmonary FibrosisThe purpose of this study is to test cough, dyspnea (shortness of breath), and quality of life (QOL) questionnaires for their accuracy, sensitivity, and ability to reliably measure the severity of cough, breathlessness, and changes in cough and disease-related quality of life over time in Idiopathic Pulmonary Fibrosis (IPF) patients. These questionnaires have been used in other types of disease, but have not all been tested and validated in patients with cough due to IPF. Our hypothesis is that worsening of cough, dyspnea, and cough-related QOL questionnaire scores will correlate with physiologic markers of IPF severity and worsening of disease. Written, valid questionnaires measuring cough, dyspnea, and QOL are important to assess the benefit of investigational drugs under development to treat patients with IPF.