Active clinical trials for "Fibrosis"

Fontan operation is currently the most common procedure performed in patients with single-ventricle physiology. This surgery allows for passive caval blood flow to the pulmonary arteries in the absence of a subpulmonary pump and therefore separating the pulmonary circulation from systemic circulation. However, late hepatic complications such as liver fibrosis, cirrhosis, or even hepatocellular carcinoma are increasingly described in patients with Fontan circulation. The Fontan associated liver disease (FALD) is related to the decreased cardiac output and elevated central venous pressure after Fontan operation. Although the prevalence of FALD is higher than 90% in patients with Fontan circulation. There are no specific medications can reverse the liver fibrosis or prevent the progression of FALD at present. Sildenafil had been used in patients after Fontan operation not only to decrease their pulmonary vascular resistance and central venous pressure, increased systemic cardiac output. Comparing with other oral pulmonary vasodilators, sildenafil is with less possibility of liver toxicity. Therefore, we will conduct a prospective, open-labeled, randomized-controlled study in at least 90 Fontan patients with age > 12 years receiving follow -up at our institute. These patients will be in accordance with the ratio of 1: 1 distribution randomized into two groups, group 1 will receive sildenafil 20 mg three times daily for 3 years and the group will not receive any pulmonary vasodilator as a control. Patients in both group will receive the examinations of liver fibrosis markers, transient ultrasound elastography (FibroScan), diffusion-weighted magnetic resonance imaging (DW-MRI) and cardiopulmonary function test at baseline, 1 year, 2 years and 3 years after initiating treatment. Comparing the results of two groups, we may further clarify the treatment effect of sildenafil on FALD in patients after Fontan procedure.

The purpose of this study is to validate and utilize a personalized medicine approach to identify potential treatments with current FDA approved CFTR modifiers for non-approved CF gene mutations. The study will perform ex vivo testing of CFTR function and current marketed CFTR modulating drugs on expanded nasal cells at Cincinnati Children's Human Nasal Epithelium (HNE) Core Laboratory. The results will be confirmed and translated into bedside care through an N of 1 trial to determine effectiveness of treatment.

Due to the radiosensitivity of the lung, radiation-induced and chemotherapy-induced pneumonitis and pulmonary fibrosis are frequent happened following cancer therapy. It not only compromise cancer treatment, but also influence patient's life qualities and even death. there are no specific treatment modalities for this treatment-induced complication. Bevasizumab (Avastin), a VEGF inhibitor, can attenuate serum high expression VEGF and pulmonary permeability, maybe effective in the control acute pulmonary fibrosis. Patients will be randomized to receive Bevasizumab (7mg/kg iv) on day one and 21, followed by Dexamethasone （10mg iv d 1-10, then 5mg iv d11-15, 2.5mg iv d16-20) or Dexamethasone alone. The thoracic CT, plasma TNF-a, IL-6, VEGF and C-reactive protein are accessed on before treatment, 24 hours after Bevasizumab, 7 days, 4 and 8 weeks. the target sample size is 30 cases.

Idiopathic pulmonary fibrosis (IPF) is a lung disease that limits the ability to breathe enough for a good workout. One way to improve the exercise training is to reduce the number of muscles being trained together. By training one leg at a time, the patient does not have to breathe as much allowing each leg a better workout. Our groundwork suggests it may work in patients with IPF. This study will help decide whether one-legged exercise training is better at improving a patient's exercise endurance compared to the usual way of exercising with both legs at the same time.

This is a Phase 1/2a randomized, double-blind, two-part, dose-ascending, multicenter study of AR-501 (gallium citrate) solution, administered via inhalation, in healthy adult and P. aeruginosa infected cystic fibrosis (CF) subjects. Phase 1 of the study in HV subjects will consist of a single-ascending-dose (SAD) cohort, followed by the HV multiple-ascending-dose (MAD) cohort. Phase 2a of the study in CF subjects will consist of a MAD study design. The study will evaluate the safety and pharmacokinetic (PK) profile of single and repeat administrations of inhaled AR-501 solution in healthy adults, and the safety, PK and efficacy of repeat administrations of inhaled AR-501 solution in P. aeruginosa infected CF subjects.

The role of Albumin in prevention and Treatment of Acute Kidney Injury (AKI) in patients with Spontaneous Bacterial Peritonitis (SBP) who are at high risk of AKI development has been clearly defined , which decreases the morbidity and mortality. But the role of Albumin in patient with SBP who are at low risk of AKI development (Serum Bilirubin <4mg/dl, Creatinine <1mg/dl at the time of presentation) has been controversial and there are no placebo controlled trials. We propose that Albumin at the standard doses is beneficial in preventing development of AKI in patients with SBP who are at low risk of AKI development.

Acute variceal upper gastrointestinal hemorrhage remains a hot potato in cirrhotic patients. The purpose of this study is to figure out whether urgent endoscopy (within 6h after gastroenterological consultation) is superior to non-urgent endoscopy (between 6h and 24h after gastroenterological consultation) in reducing re-bleeding for these patients. This is a single-centered, prospective, randomized, and controlled trial. 400 patients with suspected variceal bleeding will be randomized in a 1:1 ratio to receive endoscopic intervention either within 6h or 6-24h. Randomization is conducted by permuted block randomization stratified by age, systolic blood pressure (SBP), and pulse rate. The primary efficacy endpoint is rebleeding within 42 days after control of acute variceal bleeding. This trial will provide valuable insights into the efficacy between the urgent endoscopy group and the non-urgent endoscopy group.

The purpose of this study is to assess the efficacy of metformin to improve airway ion channel function in those with CF-related diabetes (CFRD)

Scarring of the lung, termed pulmonary fibrosis (PF), is a chronic, progressive, and usually fatal disorder. While two anti-fibrotic drugs have been approved for treating PF of unknown cause (idiopathic pulmonary fibrosis or IPF), neither drug is curative, and nearly 40% of patients stop taking the prescribed drug within a year because of side effects. The study includes the use of saracatinib, an investigational drug originally developed to treat certain types of cancers, in the treatment of IPF in a Phase 1b/2a clinical trial. The objectives of this study are to: i) evaluate the safety, tolerability, pharmacokinetics and pharmacodynamics, and to explore the efficacy of saracatinib in IPF; ii) identify biomarkers of Src kinase activity and fibrogenesis linked to pulmonary fibrosis; and iii) explore the application of these biomarkers to assess the anti-fibrotic effect of saracatinib in IPF patients

This is a first-in-human, two-part, Phase 1 study that will characterize the safety, tolerability, PK, and immunogenicity of HuL001.