A Study to Assess Pulsed Inhaled Nitric Oxide in Subjects With Pulmonary Fibrosis at Risk for Pulmonary...
Pulmonary FibrosisPulmonary HypertensionA randomized, double-blind, placebo-controlled dose escalation and verification study to assess the safety and efficacy of pulsed inhaled nitric oxide (iNO) in subjects at risk for pulmonary hypertension associated with pulmonary fibrosis on long term oxygen therapy (Part 1 and Part 2) - REBUILD
Clinical Trial of Chinese Herbal Medicine for Idiopathic Pulmonary Fibrosis (IPF)
Idiopathic Pulmonary FibrosisThis open label clinical study will be conducted in School of Chinese Medicine Clinics at The University of Hong Kong (HKU) to preliminarily determine whether treatment with the herbal formula PROLUNG could improve Idiopathic Pulmonary Fibrosis (IPF) symptoms, respiratory function and the quality of life of patients with IPF compared with pretreatment baseline. We propose to recruit 30 participants. The diagnosis and screening will be conducted by respiratory physicians. Those who meet the inclusion criteria will be referred by the expert to the PI. The PI will prescribe the formula in granule form to the participants in Traditional Chinese medicine (TCM) Clinics as usual. Patients will receive 6 months of the herbal treatment and 7 visits. Annual rate of change in forced expiratory vital capacity (FVC) will serve the primary outcome. The St. George's Respiratory Questionnaire (SGRQ) will be used to additionally assess respiratory functions. A standard questionnaire will be administered to obtain information on age, sex, marital status, education, social class, smoking behaviour and respiratory diseases. Other outcome measures include the 36-Item Short Form Survey (SF-36) and the World Health Organization Quality of Life assessment instrument (WHOQOLBREF) by each participant after enrollment.
Study of Aztreonam for Inhalation in Children With Cystic Fibrosis and New Infection of the Airways...
Pseudomonas Aeruginosa Respiratory Tract Infection/ColonizationCystic FibrosisThe primary objective of this study is to evaluate the safety and efficacy of a 14-day course versus a 28-day course of aztreonam for inhalation solution (AZLI) in pediatric participants with new onset Pseudomonas aeruginosa respiratory tract infection or colonization.
Prediction by Ultrasound of the Risk of Hepatic Cirrhosis in Cystic Fibrosis
Cystic FibrosisPancreatic InsufficiencyThe specific aims for this study are: To determine if sonographic findings predict the risk of progression of liver disease to cirrhosis by comparing cystic fibrosis subjects with heterogeneous echogenicity pattern on ultrasound to those with normal echogenicity pattern on ultrasound To develop a database and biorepository of serum, plasma, urine and DNA to aid the investigations in ascertaining the mechanisms, consequences, genetic risk factors and biomarkers for the development of cirrhosis To determine if there are differences in health related quality of life, pulmonary or nutritional status in children with cystic fibrosis who have a heterogeneous echo pattern on ultrasound compared to those who have a normal echo pattern on ultrasound To determine if Doppler velocity measurements of hepatic and splenic vessels predict an increased risk for the development of cirrhosis. To determine if cirrhosis on ultrasound progresses to portal hypertension during the study period To determine if homogeneous liver progresses to either cirrhosis or heterogeneous liver. To determine the frequency of complications of portal hypertension during follow up in those identified with cirrhosis by year 6 of the study
Effect of Lactose in Patients With Chronic Liver Disease and Minimal Hepatic Encephalopathy
Hepatic EncephalopathyLiver CirrhosisTwo groups of patients with minimal hepatic encephalopathy will be studied. The treatment group (n=17) will receive whole milk (24 g lactose) and the control group (n=17) will receive "lactose-free" milk (3.5 g of lactose) two times a day for 21 days. Clinical history, nutritional assessment, biochemical studies, psychometric tests, critical flicker frequency and a quality of life questionnaire will be performed. The patient will be assessed weekly 21 days. An external monitor will control the randomization process in order to allocate the patients into both study group and will not share the assignation codes with anyone until the end of the study.
An Exploratory Study to Assess Multiple Doses of Omalizumab in Patients With Cystic Fibrosis Complicated...
Cystic FibrosisAllergic Bronchopulmonary AspergillosisThis study will evaluate the safety and efficacy of omalizumab for the treatment of Allergic Bronchopulmonary Aspergillosis (ABPA) in patients with Cystic Fibrosis aged 12 years and older.
Miglustat / OGT 918 in the Treatment of Cystic Fibrosis
Cystic FibrosisCystic fibrosis is a genetic disease caused by mutation of the cystic fibrosis transmembrane conductance regulator (CFTR). The purpose of the study is to investigate the effects of miglustat on CFTR function in cystic fibrosis patients.
IGF-1 Therapy in Patients With Cystic Fibrosis
Cystic Fibrosis28 week pilot study to examine the efficacy of recombinant human IGF-1 on body weight and composition in adults with cystic fibrosis.
Satavaptan in the Prevention of Ascites Recurrence in Patients With Ascites Due to Cirrhosis of...
AscitesLiver CirrhosisPrimary To evaluate the efficacy of satavaptan on top of diuretic drugs in reducing the recurrence of ascites. Secondary To evaluate the tolerability and safety of satavaptan on top of diuretic drugs over a 52-week treatment period in participants with cirrhosis of the liver and recurrent ascites. The one-year double blind placebo controlled period is extended up to 2 years in a long term safety study (PASCCAL-2).
Safety Study of Autologous Stem Cell in Liver Cirrhosis
Liver CirrhosisIt is a fase I/II clinical study to evaluate feasibility, safety and kinetics of cellular therapy with bone marrow-derived mononuclear cells (ABMMC) in patients with liver cirrhosis. All the patients have moderate liver disfunction and a waiting time expectancy of liver transplantation longer than 12 months due their low MELD score. The ABMMC are labeled with 99mTc and infused through the hepatic artery. Scintigraphy is performed 2 and 24 hours after infusion. Patients are submitted to frequent clinical, laboratorial and image evaluation during the follow up period of 12 months.