Transitional Care Interventions for Patients With Cirrhosis Post-TIPS
FibrosisThe investigators conducted a randomized controlled trial to describe if multidisciplinary and patient-centered transitional care interventions for patients with cirrhosis post-TIPS can improve compliance behavior and reduce complications post-TIPS after discharge.
Evaluation of Long-Term Safety of Pirfenidone (Esbriet) in Participants With Idiopathic Pulmonary...
Idiopathic Pulmonary FibrosisThis single arm, post-authorisation study is designed to evaluate the long-term safety of pirfenidone in participants with IPF. The enrolment of participants will be completed within approximately 24 months. Participants will receive pirfenidone according to the physician discretion and will be followed for 2 years. Treating physicians will collect pre-specified data at the baseline and every 3 months thereafter, for the duration of the participants' participation in study.
Antibiotic Nephrotoxicity in Adult Patients With Cystic Fibrosis
Cystic FibrosisAdult patients with cystic fibrosis (CF) are treated with high dose antibiotics to reduce the long term damage to their lungs from infection. This would typically be with a two week course of intravenous antibiotics each time they have a chest infection (typically three to four times a year). The most effective and commonly used antibiotic in most cases is tobramycin. If this cannot be used because of previous side effects, allergy or a resistant infection then colomycin or amikacin are usually used. Each of these antibiotics are known to be toxic to both the kidneys and ear. As patients are living longer (into their forties), the total amount of these antibiotics they are receiving over their lifetime is increasing. This is now leading to increased complications such as kidney damage and hearing loss. Because of this, the investigators need to look at methods to accurately quantify damage and reduce potential kidney and hearing damage. The investigators intend to quantify kidney damage by measuring new protein markers within the urine and blood that signify kidney damage before more conventional and currently available methods are able to.In those patients treated with intravenous tobramycin the investigators will also look at an alternative method used to calculate the most appropriate dose of antibiotic for each participant. This dosing method is called 'area under the curve or AUC' dose monitoring. This method currently in clinical use in other countries is thought to more accurately reflect the most appropriate dose for each participant and thus reduce the chance of kidney and hearing problems. This 'AUC' method requires two rather than one dose level to be checked each time a dose calculation is made. Participants receiving tobramycin will be randomised to receive dosing by this method or the investigators' currently used method of 'trough' monitoring.
Relationship Between HbA1c and Chronic Glycemia in Patients With Cirrhosis
CirrhosisDiabetes Mellitus1 moreCirrhosis and advanced liver disease have been associated with an increased risk for hyperglycemia and type 2 diabetes mellitus (T2DM). The diagnostic yield of common tests used to define diabetes and insulin resistance in the general population differs significantly from the one observed in patients with liver disease. Glycosylated hemoglobin A1c (HbA1c), a reliable test to assess chronic glycemia and recommended both for the diagnosis and monitoring of T2DM, is neither accurate nor reliable in patients with cirrhosis. A validation study has not been performed to define its true usefulness in the setting of cirrhosis. The study aims to determine the level of HbA1c that better corresponds to the diagnosis of T2DM - as determined by an oral glucose tolerance test (OGTT) - and to correlate the levels of HbA1c with the average glucose over a 12-week period in patients with cirrhosis and known T2DM, in cirrhotic patients with different degrees of liver impairment as compared to patients with T2DM and no liver disease.
The Effect of Pirfenidone on Cough in Patients With Idiopathic Pulmonary Fibrosis
Idiopathic Pulmonary FibrosisIn this study we evaluate the effect of Pirfenidone on cough and quality of life in patients with idiopathic pulmonary fibrosis (IPF) that are treated with Pirfenidone in daily practice. The hypothesis is that Pirfenidone will decrease cough and increase quality of life.
Icodextrin Effects on Glucose Transporter Activation and Mediators of Fibrosis
End Stage Renal DiseaseThe time on peritoneal dialysis may be limited for a significant number of patients that use this modality of renal replacement therapy due to the inability of the peritoneal membrane to clear solutes or achieve adequate ultrafiltration, termed peritoneal membrane failure (PMF). This can be devastating for patients who have become accustomed to the quality of life provided by peritoneal dialysis and who otherwise have done well on this therapy. There is clinical evidence suggesting that icodextrin preserves the peritoneal membrane transport characteristics which may be linked to reduced cumulative glucose exposure of the peritoneal mesothelial cells. Theories to explain the role of dextrose in PMF have focused for the most part on the high intracellular concentrations of glucose without consideration to the potential pathogenic role of the glucose transporters which allow glucose entry into the cell. Experimental evidence in non-mesothelial cell lines indicate that some cellular processes that occur under high glucose conditions may not be related to intracellular glucose metabolism but to the type of glucose transporter allowing glucose entry. 3,4 However, little is known about these glucose transporters in peritoneal mesothelial cells and their potential role in the development of PMF. We hypothesize the following The presence of Sodium Glucose Co-transporter (SGLT1) on peritoneal mesothelial cells plays a role in PMF under hyperglycemic conditions. Regulation of pro-fibrotic mediators such as reactive oxidative species,transforming growth factor β, and vascular endothelial growth factor are modulated by SGLT1 activation by glucose rather than glucose metabolism or concentration. Icodextrin does not activate the SGLT1 transporter establishing a mechanism that may explain the beneficial effects of icodextrin on peritoneal membrane transport.
Role of Nox2 in CNI-induced Renal Fibrosis
Kidney DiseaseCalcineurin Inhibitors (CNI) are drugs used to suppress the immune system when a person has a solid organ transplant. Although these drugs keep the transplanted organ from being rejected they are toxic to kidneys, or nephrotoxic. CNIs cause damage, called fibrosis, to kidneys. Fibrosis is a type of scarring that occurs in kidney tissue. Fibrosis can eventually lead to kidney failure. One of the pathways that cause fibrosis is a chronic lack of oxygen to the kidney tissue called "hypoxia". There is a protein called Nox2 that may be involved in how this hypoxia happens in the kidney. The Department of Medicine-Nephrology at the University of Wisconsin is conducting a research study to see how much of the Nox2 protein is present in kidneys that may have fibrosis caused by CNIs and whether a certain type of Magnetic Resonance Imaging (MRI) can be used to tell in advance if the disease caused by CNIs is getting worse. Study hypothesis: MRI, a non-invasive technique, can be used to determine whether CNI induced kidney disease is getting worse. Additionally, the study aims to determine the role of Nox2 in CNI nephrotoxicity.
Cough Efficiency in Cystic Fibrosis
Cystic FibrosisThe major causes of morbidity and mortality in Cystic Fibrosis (CF) are linked to the process of chronic inflammatory of the airway, leading to the progressive damage of the small bronchioles and subsequently to the proximal bronchi. A connection between weaknesses of respiratory muscles in CF and deficits of CFTR in the muscle has been established. Insufficient cough in CF patients may advance re-current respiratory infections. A voluntary cough flow volume (C-FVC) profile incorporates the characteristics of the forced expiratory flow volume curve (FE-VC). The study aims to explore the correspondence of voluntary cough-flow-volume and maximum expiratory flow-volume maneuvers in relation to disease complications in CF patients.
Assessment Of Liver and Spleen Fibrosis in Patients With Gaucher Disease Using Fibroscan
FibrosisThe investigated cohort will examine liver and spleen fibrosis in patients with Gaucher disease divided into two groups, naive GD patients and GD patients treated with ERT. As liver biopsy in these patients not recommended because the risk of bleeding using Fibroscan is a safe with diagnostic accuracy regarding the liver (& Spleen) fibrosis. Estimating spleen fibrosis is an innovative approach in liver disease and Gaucher. The evaluation of fibrosis with this new and safe method could avoid complications antiinvasive procedure in GD patients. The addition of fibrosis biomarkers will help for patients score evaluation. The finding of liver and spleen stiffness will be evaluated in native and ERT treated Gaucher patients in order to assess ERT effect on fibrosis. The Aims are: 1) To assess liver and spleen stiffness measurement using fibroscan and evaluate liver and spleen fibrosis in patients with GD. 2) To compare the elastography in two cohorts of GD patients: ERT treated and naïve GD patients and two control groups of patients: healthy and Non Alcoholic Steatohepatitis (NASH) patients. 3) To correlate the elastography findings with clinical and laboratory data in the four patient groups focusing on Gaucher disease manifestations and GD severity. To compare the elastography in GD naïve and ERT treated patients.
68Ga-BMV101 PET/CT in Patients With Idiopathic Pulmonary Fibrosis
Idiopathic Pulmonary FibrosisInflammation mediated by macrophage infiltration plays a vital role in a diverse range of physiological conditions. In particular, recent evidence suggests this type of macrophage response is important for the disease pathology of pulmonary fibrosis. Because cysteine cathepsins are proteases that are highly expressed in antigen presenting cells such as macrophages, they serve as promising biomarkers. Employing non-invasive imaging agents 68Ga-BMV101 that specifically recognize cysteine proteases in immune cells has the potential to not only aid early detection but also significantly aid efforts to monitor progression and patient response to therapy.