
Volatile Organic Compounds in Cystic Fibrosis
Cystic FibrosisPseudomonas AeruginosaThis experiment is designed to test the effectiveness of a new electronic nose device, which allows a non-invasive breath test for markers of lower respiratory tract infection, which may predict the probability of bacterial organisms in the lower respiratory tract. It consists of: A breath collection apparatus for collection of volatile organic compounds in breath onto a sorbent trap and Tedlar bag, as well as for the collection of a separate sample of room air. Analysis of the volatile organic compounds in breath and room air by short acoustic wave/gas chromatography. Interpretation of the volatile organic compounds with a proprietary algorithm in order to predict the probability of lower respiratory tract colonization and infection. This study will test the hypothesis that the investigators can identify the presence of Pseudomonas aeruginosa by sampling the "head space" above culture media of sputum provided by patients with cystic fibrosis. This study will test the additional hypothesis that the investigators can identify the presence of Pseudomonas aeruginosa by sampling exhaled breath from the patient providing the sputum.

Rare Genetic Disorders of the Breathing Airways
Kartagener SyndromeCystic Fibrosis2 moreMucociliary clearance, in which mucus secretions are cleared from the breathing airways, is the primary defense mechanism for the lungs. Inhaled particles, including microbes that can cause infections, are normally entrapped in mucus on the airway surfaces and then cleared out by the coordinated action of tiny hair-like structures called cilia. Individuals with primary ciliary dyskinesia, variant cystic fibrosis, and pseudohypoaldosteronism have defective mucociliary clearance. The purpose of this study is to collect clinical and genetic information about these three airway diseases to improve current diagnostic procedures.

Evaluation of a Decision Aid for Adult Cystic Fibrosis Patients Considering Bilateral Lung Transplantation...
Cystic FibrosisIn 2006, the International Patient Decision Aids Collaboration (IPDAS) reached agreement on criteria for evaluation. Accordingly, the primary outcome is 'decision quality' 3 weeks after using the decision aid. Decision quality is defined as the extent to which a patient decision aid improves the match between the chosen option and the features that matter most to the informed patient. Measures include: a) patients' knowledge of essential facts [options, benefits, harms, and outcome probabilities]; and b. the congruence between the option chosen and patients' informed values regarding benefits and risks. In the past twenty years lung transplantation has become the most widely accepted option of treating cystic fibrosis patients with severe lung disease. Lung transplantation can be a good experience for many patients, improving their quality of life and their survival. However there are potential risks of lung transplantation including infection, organ rejection and early death. Cystic fibrosis patients are often faced with making a choice of whether to be referred for lung transplantation when they are very sick and there is the immediate need to survive. Our group has developed a tool called a decision aid which we hope will assist the patient and family in making this choice. The decision aid guides the patient through a series of steps where they weigh the benefits and risks of being referred for lung transplantation and the benefits and risks of receiving 'usual care' without the option of referral.

Endoscopic Band Ligation (EBL) Versus Endoscopic Band Ligation and Propranolol for the Prevention...
CirrhosisVariceal BleedingBoth propranolol and endoscopic band ligation (EBL) are effective for prevention of variceal rebleeding. Recently several studies compared the efficacy of EBL alone and with a combination of propranolol and EBL. However, the results of recent studies showed discrepancy. This study is performed to compare the efficacy and safety of EBL alone and EBL combined with propranolol in patients without previous history of endoscopic variceal treatment.

Assessment of Fat Free Mass Index and Its Impact on Health in Children With Cystic Fibrosis
Cystic FibrosisThe purpose of this study is to find out whether measurement of body composition has a prognostic value on clinical and overall outcome in pediatric patients with cystic fibrosis.

Evaluation of Liver Fibrosis Following Surgical-induced Weight Loss
ObesityObesity is a frequent disease with an increased prevalence, estimated to 12.4% in France (OBEPI study 2006). Non alcoholic fatty liver disease (NAFLD) and non alcoholic steatohepatitis (NASH) both complicate morbid obesity and could be responsible for the development of liver fibrosis and consequently of liver cirrhosis. Bariatric surgery has been widely used in obese patients to reduce their weight but also to decrease morbidity conditions related to obesity. It could be involved in an improvement in liver lesions observed in obese patients. To evaluate the severity of liver lesions, the gold standard is pathological examination of the liver using percutaneous or surgical biopsies, with a scoring called Fibrosis score stage. Alternative less invasive techniques have been developed to evaluate liver lesions. These tests are commonly used in clinical hepatology and consisted of serum levels determination of fibrosis markers (Fibrotest, Transthyretin). The aim of this study is to evaluate the evolution of fibrosis scores during weight loss in a population of patients operated on for morbid obesity ( BMI > 40kg/m2 or BMI> 35kg/m2 with comorbidity). The primary end point is to evaluate the concordance between Fibrotest and transthyretin serum levels. The number of patients has been calculated on the basis of a concordance ( Kendall test) superior to 70% in patients with grade F1 fibrosis that represents about 40% of morbid obese patients. A total of 255 patients has to be enrolled in this study. According to the number of patients operated on in the 3 centres (>150/year), the time for recruitment is 12 months. Secondary end points will evaluate in these patients the excess weight loss, the improvement in fibrosis liver tests and the correlation with improvement in other obesity- related conditions, according to surgical procedures.

Screening of Hepatocellular Carcinoma in Patients With Compensated Cirrhosis
Compensated CirrhosisLiver carcinoma is becoming the main complication of cirrhosis. Treatment of symptomatic or large tumors is disappointing. Regular ultrasonographic screening of small (curable) tumors is currently recommended, but the best periodicity is unknown.This randomized trial is aimed to compare 6-month (current recommendation) and 3-month ultrasonographic screenings.

Effects of a Comprehensive Patient-centered Health Service in Cystic Fibrosis (VEMSE-CF)
Cystic FibrosisFor the Project VEMSE-CF, a comprehensive patient-centered outpatient health service model was developed offering focused interventions in different areas. These included patient education, as well as nutritional and exercise counselling. Special emphasis was given to the psycho-social services. The implementation was supported by a case manager. The model was implemented in three German CF-Centers. For evaluation, Patient data from 13 additional German CF-Centers offering standard care was used as comparison. In total, 153 patients in the Intervention Group and 163 control patients aged 5-52 years contributed data. The primary endpoint was the number of BMI- and FEV1-measurements over 24 months. Secondary endpoints included mortality, lung transplantation, FEV1, BMI, adherence to therapy, Quality of life, and mental stress.

Circulating P3NP and Cardiovascular Events in Hemodialysis Patients
Cardiovascular EventsCardiac FibrosisThis study evaluates whether circulating levels of aminoterminal propeptide of type III procollagen (PIIINP) can predict the cardiovascular events in hemodialysis patients.

Clinical Impacts of Achieving SVR in Patients With Advanced Hepatic Fibrosis Related to Chronic...
HCC in Chronic HCV Patients With Advanced Liver FibrosisChronic HCV infection has relatively slow rate of progression. Liver fibrosis is the main sequlae and usually progressed to cirrhosis after long period (10 to 20 years) [6]. Once cirrhosis is established the disease progression remains unpredictable: cirrhosis can remain indolent for many years in some patients whilst progressing in others to HCC, hepatic decompensation and death. Overall once cirrhosis has developed there is a 1-5% annual risk of HCC and a 3-6% annual risk of hepatic decompensation. Following an episode of decompensation the risk of death in the following year is between 15% and 20% Treatment of chronic HCV has been dramatically changed in the last few years with introduction of direct acting antivirals (DAAs). The new therapies with excellent safety profiles, shorter duration of therapy and marked higher efficacy can be even used in patients with advanced fibrosis and cirrhosis