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This program is to provide expanded access to aztreonam lysine for inhalation (AZLI) prior to its commercial availability to patients with cystic fibrosis (CF) and chronic P. aeruginosa airway infection who have limited treatment options and are at risk for disease progression.
Published on BioPortfolio: 2014-08-27T03:18:52-0400
The primary objective of this program is to provide expanded access to aztreonam lysine for inhalation (AZLI) 75 mg prior to its commercial availability to patients with cystic fibrosis (C...
The primary objective of this study is to assess the safety and efficacy of a 28-day course of aztreonam lysine for inhalation (AZLI) in patients with cystic fibrosis (CF), mild lung disea...
The aim of this observational trial is to evaluate the pulmonary function in cystic fibrosis patients that have been treated with inhaled aztreonam lysine comparing the previous 12 months ...
The purpose of this research study is to see if an experimental drug called Aztreonam for Inhalation Solution is safe and effective to treat Burkholderia lung infections in patients with C...
In cystic fibrosis, there is a critical need for better predictors of treatment response. The investigators have identified a panel of white blood cell biomarkers which can be directly mea...
Cystic fibrosis is the most prevalent metabolic chronic disease in Caucasian children in the Europe. Cystic fibrosis has seen approximately 1 in 3000 births in northern European countries. Birth preva...
The risk of vitamin E deficiency is of primary concern in cystic fibrosis patients. However, early diagnosis and routine vitamin E supplementation can lead to its normal or even high levels. In the pr...
In healthy lungs, epithelial sodium channel (ENaC) is regulated by short, palate, lung, and nasal clone 1 (SPLUNC1). In cystic fibrosis (CF), ENaC is hyperactivated in part due to a loss of SPLUNC1 fu...
In vitro activity of β-lactams in combination with avibactam against multidrug-resistant Pseudomonas aeruginosa, Stenotrophomonas maltophilia and Achromobacter xylosoxidans isolates from patients with cystic fibrosis.
The in vitro activity of anti-pseudomonal β-lactams in combination with avibactam was evaluated against 54 multidrug-resistant non-fermenting Gram-negative bacilli isolated from cystic fibrosis patie...
An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION.
A chloride channel that regulates secretion in many exocrine tissues. Abnormalities in the CFTR gene have been shown to cause cystic fibrosis. (Hum Genet 1994;93(4):364-8)
A strain of mice widely studied as a model for cystic fibrosis. These mice are generated from embryonic stem cells in which the CFTR (cystic fibrosis transmembrane conductance regulator) gene is inactivated by gene targeting. As a result, all mice have one copy of this altered gene in all their tissues. Mice homozygous for the disrupted gene exhibit many features common to young cystic fibrosis patients, including failure to thrive, meconium ileus, and alteration of mucous and serous glands.
A species of STENOTROPHOMONAS, formerly called Xanthomonas maltophilia, which reduces nitrate. It is a cause of hospital-acquired ocular and lung infections, especially in those patients with cystic fibrosis and those who are immunosuppressed.
Intestinal obstruction caused by congealed MECONIUM in the distal ILEUM and CECUM. It presents shortly after birth as a failure to pass meconium and frequently occurs in infants with CYSTIC FIBROSIS.
Antiretroviral Therapy Clostridium Difficile Ebola HIV & AIDS Infectious Diseases Influenza Malaria Measles Sepsis Swine Flu Tropical Medicine Tuberculosis Infectious diseases are caused by pathogenic...
Affecting over 8,500 people in the UK, Cystic Fibrosis (CF) is one of the UK's most common life-threatening inherited diseases. Around half of the CF population can expect to live over 38 years, although improvements in treatments mean a baby born ...