View clinical trials related to Cystic Fibrosis.
Filter by:CF patients are at risk for hepatic disease. Vaccination is recommended to all CF patients according to European consensus. The aim of the study is to vaccinate as many patients as possible and to follow up whether immunization has been complete.
Methicillin-susceptible (MSSA) and Methicillin-resistant (MRSA) Staphylococcus aureus (SA) are two of the most important infectious pathogens in CF, with 69% of CF patients having lung infection with MSSA or MRSA in the last year. Wolter and co-workers recently demonstrated that a specific morphologic subtype of MSSA and MRSA, small-colony variant Staph aureus (SCV-SA), is associated with greater decline in lung function and worse clinical outcomes. SCV-SA is already recognized for its ability to contribute to persistent infection, likely due to SCV-SA's ability for intracellular growth, as well as its increased antibiotic resistance compared to normal-colony SA. To investigate the epidemiology and clinical significance of SCV-SA in CF, and explore the hypothesis that SCV-SA may require unique antibiotic treatment strategies to optimize clinical response, the investigators will perform the following: 1. Characterize the epidemiology of SCV-SA infection in both an adult and pediatric CF population and investigate the clinical significance of SCV-SA infection in CF by comparing clinical characteristics and outcomes of CF patients with SCV-SA compared to those with to normal-colony MSSA/MRSA. 2. Characterize the unique microbiologic characteristics of SCV-SA infection in CF by evaluating antibiotic susceptibility profiles and molecular characteristics of SCV-SA in a two large CF patient populations. 3. Perform a 16-patient pilot study of a novel treatment for SCV-SA infection in CF, utilizing low dose rifampin in combination with standard anti-SA antibiotics. These investigations will delineate the role of SCV-SA as a pathogen in CF and provide guidance to optimize treatment strategies of MSSA/MRSA CF lung infection.
The purpose of this study is to compare the incidence of nausea and vomiting following short intermittent versus prolonged intermittent infusion of meropenem.
The current dream in CF research is to discover safe drugs that correct the basic defect and prevent lung disease, allowing patients without significant lung damage to live nearly normal lives with a dramatic increase in life expectancy and without the burden of current treatment. The compound VX-770 (Ivacaftor Ò) is hoped to be the first milestone along this way. Progression of lung disease is now so gradual in many centres that sensitive indicators of early lung disease (small airways disease) are critically needed to assess the effects of such new treatments. In this context, assessment of ventilation inhomogeneity by the measurement called Lung clearance index (LCI) seems to be the most promising tool. However, to get approval by health authorities, new measures used in drug evaluation need to fulfill strict criteria. For LCI, the investigators still need to prove its long term significance: How well does the LCI measurement predict the long term lung disease course? Therefore, in this study the investigators want to measure LCI at baseline in a large patient cohort and establish how well it predicts the patients' disease course over the next 2 years.
Null hypotheses: zoledronic acid does not improve bone density in cystic fibrosis. Low bone mineral density (osteoporosis) is prevalent in adults with cystic fibrosis (CF); they have an increased rate of bone fractures in comparison to the general population. CF patients start to lose bone density in adolescence/early adulthood due to an imbalance in bone breakdown and formation. Predicted survival for patients with CF has increased from 16 years in 1970 to 36.5 years in 2009 which has resulted in an increase in comorbidities associated with increased longevity in CF e.g. decreased bone density. Oral and intravenous bisphophosphonates are known to increase bone density in CF; the current licensed oral preparations require daily or weekly dosing which are difficult to maintain. Zoledronate, which is licensed for use, is administered intravenously once a year which should be easier to administer. The current evidence relates to its use in other disease groups e.g. glucocorticoid induced osteoporosis and oncology. The purpose of this study is to ascertain its efficacy in cystic fibrosis.
The purpose of this study is to determine the pharmacokinetics and safety of inhaled vancomycin in patients with cystic fibrosis.
Using CFF registry data, this analysis will: describe patterns of time to TIS initiation from first year of TIS eligibility, estimate the increased risk of death attributable to each year of delayed TIS initiation, and investigate TIS effects across study centers.
A major factor in the respiratory health of Cystic Fibrosis (CF) subjects is the prevalence of chronic Pseudomonas aeruginosa infections. The Pseudomonas aeruginosa infection rate in CF patients increases with age and by age 18 years approximately 85% of CF patients in the US are infected. Liposomal amikacin for inhalation (Arikace™) was developed as a possible treatment for chronic infection due to Pseudomonas aeruginosa in CF patients. The purpose of this double-blind, placebo controlled study is to determine whether Arikace™ is effective in treating chronic lung infections caused by Pseudomonas aeruginosa in Cystic Fibrosis subjects. The study will enroll approximately 300 subjects in clinics in the US, Canada, Europe, Australia and New Zealand. Subjects will be randomized to 590 mg Arikace™ or placebo and will receive treatment for 28 days followed by a 56 day safety follow-up period. The subjects will be required to visit the clinic 8 times (including the Screening visit) over a period of approximately 3 months. No overnight stays at the clinic will be required. At the completion of the TR02-109 protocol, subjects who have consented and meet study safety criteria may enroll in the long-term, open-label, multi-cycle extension study of 590 mg of Arikace™ (under a separate protocol TR02-110).
The primary objective of this study is to measure efficacy of our new protocol by monitoring the results of our routine respiratory cultures at the end of the new standard treatment, and during routine visits for 1 year from initiation of therapy for Staphylococcus aureus. The secondary objective will include determining the clinical course (pulmonary exacerbations, antibiotic use, hospitalizations, pulmonary function tests) of patients who underwent the protocol.
Cystic Fibrosis (CF), the most common inherited disease in Caucasians, is characterized by chronic pulmonary inflammation and progressive loss of gas exchange units that eventually results in respiratory failure. There is strong evidence that, in CF, abnormally low perfusion carries a high risk of death independent from the presence of pulmonary hypertension. However, the evolution of pulmonary vascular disease in CF and how it might contribute to the rate of decline in lung function is not known. Our knowledge remains limited to the results of old observational studies which concluded that the major causes of pulmonary vascular remodeling and hypertension in CF are hypoxic respiratory failure and destruction of lung tissue. Our recent data obtained by state-of-the-art Magnetic Resonance Imaging (MRI) of the pulmonary circulation, challenges the existing paradigm. We demonstrate that in the absence of hypoxia, significant changes in pulmonary perfusion and in surrogate measures of vascular resistance as well as in collateral blood flow begin early in the course of CF. Newly developed therapeutics have altered dramatically the course of patients suffering from pulmonary vascular disease. Through this 8 week trial, we will examine by Magnetic Resonance Imaging the effect of Sildenafil on pulmonary perfusion and systemic vascularization of the lungs in subjects with mild to moderate disease.