Active clinical trials for "Fibrosis"

Cystic fibrosis (CF) is characterized by airway inflammation and infection leading to progressive destruction of lungs. One of the most important abnormalities in CF is an abnormal processing of the mutated CFTR protein through the endoplasmic reticulum that causes abnormal location or even absence of the protein at the apical plasma membrane of airway epithelial cells. This abnormality results in a marked dehydration of the airway surface fluid, decreased mucus transport and airway obstruction. Nevertheless, the events that occur very early during the progression of the disease at the airway level in infants are not known. At cellular level, it has also been reported that the CFTR expression and localization could be related to the differentiation state of the airway epithelium. Furthermore, it has been reported that gap junctions could be involved in dysregulate inflammation process. In CF infants, many answers are still lacking. For a better understanding of the early stages of cystic fibrosis, it is of major interest to study respiratory epithelial cells obtained as early as possible. In 15 CF infants and 15 control infants, a nasal brushing will be performed by means of a soft sterile cytology brush. Samples will be used for cytological and functional studies: ciliary beating frequency, cAMP-dependent chloride efflux, potassium efflux, tight and gap junctions functionalities. These studies will be done in basal conditions and will be repeated after activation of the nasal epithelial cells by the bacteria, such as Staphylococcus aureus, which can be found very early in the course of CF disease.

The RIAL study aims to investigate whether non-invasive measurement of liver fat, iron content and fibrosis are as accurate as liver biopsy specimens in determining if patients have non-alcoholic fatty liver disease (NAFLD) or steatohepatitis (NASH), or other suspected liver disease. Currently, the gold-standard for the diagnosis and staging of liver disease is a liver biopsy. In this study, consecutive patients will be offered a multiparametric MR scan to assess their liver while they await a liver biopsy. Study time-frame: The scan will be performed in the 6-week period before their biopsy, and results will be compared to biopsy findings. results will be presented at the end of the study when MR data outcomes are compared to gold-standard biopsy dat. Participants will only have to attend one study visit to participate - there will be no patient follow-up.

The pathogenesis of idiopathic pulmonary fibrosis (IPF) is incompletely understood but recurrent epithelial injury occurs which evokes the coagulation cascade. Thrombin is produced as a result and is over expressed in IPF patients, so may be important in propagating disease activity. We aim to recruit patients with IPF and then complete FDG (18F-2-fluoro-2-deoxy-D-glucose fluorodeoxyglucose) PET (positron emission tomography) scans pre and post manipulation of the coagulation cascade to assess the role of this biological pathway in disease activity. Previous studies from our institution have demonstrated increased FDG avidity in the lungs of patients with IPF (assessed using FDG PET scans) but to date the cells and pathways responsible for this signal have not been identified and thus need further exploration.

The aim of this study is to evaluate the diagnostic performance of FEUrea for the differential diagnosis of AKI in patients with cirrhosis and ascites Specifically, the ability of FEUrea to distinguish between ATN versus Pre renal azotemia and HRS.

This is a national, investigator-initiated, multicenter, prospective, observational, web-based registry in hospitalized patients with cirrhosis across China. The overarching aim of this study is to investigate the epidemiology and clinical impact of bacterial/fungal infections in hospitalized patients with liver cirrhosis in China within the collaborative network. We also aimed to build up the national prospective cohort of hospitalized cirrhosis in China to stand in the future for the backbone of various research programs focused on infection, other complications of cirrhosis, organ failure, the ACLF syndrome, end-stage liver disease and beyond.

A prospective single center observational cohort of patients with cystic fibrosis to determine whether adequate serum levels of posaconazole, after administration of the newer modified release once daily oral formulation, can be achieved.

ARDS is a severe disease in ICU, and could induce high mortality. Glycocalyx is an important matrix construction which covers endothelial cells, it could protect endothelial cells injury and the glycocalyx biomarkers could predict pulmonary injury. Decorin is a member of the small leucine-rich proteoglycan (SLRP) family, contains a single glycosaminoglycan (GAG) chain and a core protein with 12 leucine-rich repeats. In the previous study, decorin is associated with cardiac and liver fibrosis, however, the effects of decorin on ARDS pulmonary fibrosis have not been clarified.

The investigators assess the prevalence of airway disease associated with idiopathic pulmonary fibrosis in Korea, and evaluate the effect of these airway diseases on the symptoms and quality of life of patients with idiopathic pulmonary fibrosis.

Patients with cirrhosis of viral etiology (HCV/HBV); Patients with cirrhosis of any other etiology (alcohol, idiopatic, autoimmune). Planned Number of cirrhotic subjects 200 patients Inclusion Criteria Subjects (18 yr old) with liver cirrhosis of any etiology, Exclusion Criteria All patients should not have hepatocellular carcinoma or other malignant tumors, they should not be treated with anticoagulant / antiplatelet agents, not affected by PVT already diagnosed and not suffering from congenital coagulation disorders (haemophilia A / B, von disease Willebrand, another congenital deficiency of coagulation factors) or severe thrombocytopenia (<30,000 Plt / μL). Subject has participated in another clinical study within 30 days prior to study enrollment or is scheduled to participate in another clinical study on cirrhosis Primary Objective To describe the prospective modification of ADAMTS-13 level and other coagulation variables (e.g. FVIII, VWF:Ag/VWF:act) in cirrhotic patients during 18 months from the enrolment and to verify their predictive role as biomarker of development of portal vein thrombosis (PVT) Secondary Objectives To describe prospectively the modification of ADAMTS-13 level as a function of the etiology of cirrhosis Statistical analysis The total duration of the study will be of 12 months. The sample size of 200 subjects will be selected as a feasible number of patients to be recruited in a period of six months. The patients will be consecutively enrolled and followed for 18 months. As a result, in a follow up period of 18 months about 20-25 cases of PVT are expected. Continuous variables will be expressed as means ± standard deviations. In addition to descriptive statistics (location parameters), univariate analysis will be performed on each parameter and development of PVT during the follow up period. In previous observational studies both 1) a reduced PV flow [prospectively] and 2) a reduction of ADAMTS-13 are significantly associated with PVT. These associations will be investigated prospectively and analyzed simultaneously by a multivariate analysis and ROC curve to establish the sensitivity and specificity of these parameters as predictors of PVT development. Analyses will be performed using available data

There have been substantial advances in care for individuals with CF, including improved nutrition and respiratory care. Women with CF are now likely to survive into adulthood with a median predicted survival of 49.7 years in 2012, which have resulted in increasingly normal lifestyles, including successful pregnancies in women with CF. The number of pregnancies in CF women has increased as reported by the 2013 Annual Data Report from the French CF Registry (6 pregnancies in 1992 to 53 in 2012). Pregnancy adds many physiological stresses to the body, particularly on pulmonary function (decrease in residual volume, expiratory reserve capacity) and nutritional status (increase in nutritional needs). In women with CF, these added stresses could theoretically affect survival, with difficulties to maintain adequate nutrition and an unpredictable effect on lung function. A number of studies published over the past 15 years have attempted to clarify the risk of pregnancy in women with CF to guide clinicians and women. Many authors compared the outcomes of pregnant CF women to non-pregnant CF women regardless of nutritional status, lung function, diabetes mellitus, bacteriological colonization. In literature, pregnancy was possible and well tolerated in CF women with mild disease if associated with more intensive monitoring and aggressive treatment during pregnancy, with particular emphasis on nutrition and weight gain. CF women have an increased likelihood of receiving treatment for diabetes both during and after pregnancy. Furthermore diabetes mellitus has been associated with more severe pulmonary disease, more frequent pulmonary exacerbations, poorer nutritional status and a reduced life expectancy. Pre-pregnancy diabetes mellitus is associated with a poorer prognosis for pregnancy in general population. Severe forms of CF, including pre-pregnancy diabetes mellitus might as well do badly with accelerated decline after pregnancy. To determine the effect of pre-pregnancy diabetes on maternal decline after pregnancy, we will compare FEV1 and BMI rates of decline, during a two-years follow-up period after pregnancies, reported in the French CF Registry, according to their diabetic status.