|2.||Lung Diseases (Lung Disease)
|4.||Asthma (Bronchial Asthma)
|5.||Chronic Obstructive Pulmonary Disease (COPD)
|1.||Ratjen, Felix: 63 articles (11/2015 - 02/2002)|
|2.||Konstan, Michael W: 48 articles (11/2015 - 05/2002)|
|3.||Elborn, J Stuart: 41 articles (12/2015 - 09/2003)|
|4.||McElvaney, Noel G: 36 articles (12/2015 - 06/2003)|
|5.||Boucher, Richard C: 35 articles (12/2015 - 02/2002)|
|6.||Zeitlin, Pamela L: 35 articles (01/2015 - 03/2002)|
|7.||Amaral, Margarida D: 34 articles (11/2015 - 09/2002)|
|8.||Greene, Catherine M: 32 articles (12/2015 - 06/2003)|
|9.||Accurso, Frank J: 32 articles (11/2015 - 03/2002)|
|10.||Becq, Frédéric: 31 articles (10/2015 - 09/2002)|
|1.||Tobramycin (Nebcin)FDA LinkGeneric
01/01/2007 - "Delivery of tobramycin by inhalation to the lungs of patients with cystic fibrosis (CF) who are infected with Pseudomonas aeruginosa has been proven to be effective and safe. "
01/01/2000 - "Phase III trials of the aerosolized delivery of tobramycin to cystic fibrosis (CF) patients demonstrated a significant improvement in pulmonary function and in sputum bacterial density. "
01/01/2015 - "In vitro efficacy of high-dose tobramycin against Burkholderia cepacia complex and Stenotrophomonas maltophilia isolates from cystic fibrosis patients."
03/15/2003 - "We conclude that 28 days of tobramycin solution for inhalation of 300 mg twice daily is safe and effective for significant reduction of lower airway Pa density in young children with cystic fibrosis."
06/01/2014 - "Clinical use of tobramycin inhalation solution (TOBI®) shows sustained improvement in FEV1 in cystic fibrosis."
|2.||Anti-Bacterial Agents (Antibiotics)IBA
01/01/2015 - "Do veterinary antibiotics have efficacy against highly resistant Gram-negative pathogens from patients with cystic fibrosis?"
07/01/2001 - "Most children with cystic fibrosis (CF) feel better and display more energy after a course of intravenous antibiotics (IVABs), but this is not always reflected by a satisfactory improvement in lung function. "
01/01/2003 - "Although it is difficult to separate the beneficial effects of the various aspects of cystic fibrosis treatment, longer survival and improved quality of life have been associated with the advance of antibiotics varieties. "
02/01/1999 - "Are antipseudomonal antibiotics really beneficial in acute respiratory exacerbations of cystic fibrosis?"
01/01/2014 - "Once- and three-times daily aminoglycoside antibiotics appear to be equally effective in the treatment of pulmonary exacerbations of cystic fibrosis. "
|3.||human DNASE1 protein (dornase alfa)FDA Link
01/01/2001 - "Most respiratory complications in cystic fibrosis (CF) arise from abnormally viscid mucus, and rhDNase has shown to be effective in enhancing mucous clearance. "
05/01/2001 - "The aim of this study was to evaluate the ability of high-resolution computerized tomography (HRCT) of the chest and chest x-rays (CXR) to determine efficacy of inhaled recombinant human DNase (rhDNase) in cystic fibrosis (CF) patients younger than 5 years of age. "
08/01/1995 - "Treatment with either rhDNase or high-frequency oscillation has been shown to be effective in improving the physical and transport properties of airway secretions in cystic fibrosis (CF). "
12/01/2005 - "Nasally inhaled dornase alfa can be effective in patients with cystic fibrosis and sinonasal disease who do not respond to conventional therapy after surgical treatment. "
06/01/1999 - "Patients with cystic fibrosis (CF) receiving dornase-alfa had improved pulmonary function relative to a control group in a large randomized phase III controlled study. "
|4.||Azithromycin (Zithromax)FDA LinkGeneric
04/01/2005 - "Azithromycin appears to be safe and effective, and is thus the most promising antiinflammatory therapy available for patients with cystic fibrosis. "
09/01/2009 - "Although long-term use of azithromycin has shown a significant clinical improvement for patients with cystic fibrosis (CF), its long-term effect on the susceptibility of commensal flora within CF airways has not yet been examined. "
11/01/2004 - "The evidence for the clinical benefit of azithromycin in cystic fibrosis has been summarized in a Cochrane review in which a meta-analysis confirmed a significant improvement in forced expiratory volume in 1 second among the 286 pooled participants. "
09/28/2002 - "The macrolide antibiotic azithromycin has anti-inflammatory properties potentially beneficial in cystic fibrosis. "
11/01/2007 - "Azithromycin has antiinflammatory properties and is effective in cystic fibrosis (CF) patients. "
|5.||Ceftazidime (Fortum)FDA LinkGeneric
12/01/2000 - "Continuous infusion of ceftazidime with a portable pump is as effective as thrice-a-day bolus in cystic fibrosis children."
05/01/1986 - "Ceftazidime appears to be effective and safe, alone or associated with an aminoglycoside, in the treatment of acute exacerbation in cystic fibrosis. "
09/01/2009 - "The present multicenter, randomized crossover study compared the safety and efficacy of continuous infusion with those of short infusions of ceftazidime in patients with cystic fibrosis. "
07/01/1988 - "Influence of the glomerular filtration rate on renal clearance of ceftazidime in cystic fibrosis."
08/01/1983 - "The pharmacokinetics and clinical efficacy of ceftazidime, a cephalosporin with activity against Pseudomonas aeruginosa, were studied in children with cystic fibrosis. "
|6.||DNA (Deoxyribonucleic Acid)IBA
05/01/1993 - "Rapid cycle DNA amplification is a polymerase chain reaction technique with improved product specificity and cycle times of 20-60 s, allowing complete 30-cycle reactions in 10-30 min. The presence or absence of the delta F508 deletion and wild-type allele was determined in 104 cystic fibrosis patients by rapid cycle DNA amplification. "
01/01/1989 - "Effectiveness of prevention of cystic fibrosis by artificial insemination by donor can be markedly improved by DNA-analysis of sperm donors."
02/01/1990 - "The ability to detect the common mutation causing cystic fibrosis represents a major improvement in prenatal diagnosis and heterozygote detection, particularly in families in which no DNA sample is available from the affected child, and provides an improved method of testing for spouses of carriers of cystic fibrosis. "
01/20/1998 - "GTAB1001: A Double-Blind, Placebo Controlled, Dose Ranging Study to Evaluate the Safety and Biological Efficacy of the Lipid-DNA Complex GR213487B in the Nasal Epithelium of Adult Patients with Cystic Fibrosis. "
01/20/1998 - "A double-blind, placebo controlled, dose ranging study to evaluate the safety and biological efficacy of the lipid-DNA complex GR213487B in the nasal epithelium of adult patients with cystic fibrosis."
|7.||Ciprofloxacin (Cipro)FDA LinkGeneric
03/01/1997 - "Ciprofloxacin, an orally-absorbed fluoroquinolone is effective against multiply resistant Pseudomonas aeruginosa in cystic fibrosis patients. "
07/01/1988 - "Ciprofloxacin appears to be effective in patients with cystic fibrosis who are experiencing pulmonary exacerbations associated with susceptible bacterial pathogens."
04/27/1987 - "Ciprofloxacin monotherapy is effective for the acute treatment of cystic fibrosis patients. "
06/01/1997 - "Ciprofloxacin thus appears to be safe and effective for use in young patients with bronchopulmonary exacerbation of cystic fibrosis."
01/01/1997 - "The efficacy of ciprofloxacin was estimated for the last 3 years in the treatment of 139 children with mucoviscidosis. "
01/01/2011 - "This use has been well established both in diffuse panbronchiolitis and in cystic fibrosis. "
01/01/2009 - "Prolonged macrolide antibiotic therapy at low doses improves clinical outcome in patients affected with diffuse panbronchiolitis and cystic fibrosis. "
08/01/2008 - "Long-term, low-dose macrolide therapy has been proven to improve survival in patients with diffuse panbronchiolitis and cystic fibrosis, although the mechanisms by which it does so remain unknown. "
05/01/2007 - "Pseudomonas aeruginosa is one of the most important pathogens in patients with chronic airway conditions, such as cystic fibrosis and diffuse panbronchiolitis. "
02/01/2006 - "They are frequently used in the treatment of diffuse panbronchiolitis and cystic fibrosis (CF). "
|9.||Cystic Fibrosis Transmembrane Conductance Regulator (Protein, CFTR)IBA
10/01/2015 - "While a major target in cystic fibrosis (CF) research in recent years has been the development of corrector and potentiator drugs targeting the cystic fibrosis transmembrane conductance regulator (CFTR) protein, these therapies have not yet proven robust enough to replace or eliminate other therapies that have demonstrated improved health outcomes and quality of life in patients with CF. "
10/01/2011 - "Modulator compounds intended to overcome disease-causing mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) show significant promise in clinical testing for cystic fibrosis. "
06/01/2005 - "Strategies to improve mutant cystic fibrosis transmembrane conductance regulator function or to bypass mutant cystic fibrosis transmembrane conductance regulator function hold great promise for development of novel therapies aimed at correcting the underlying pathophysiology of cystic fibrosis."
11/01/2015 - "Treatment of cystic fibrosis patients with the cystic fibrosis transmembrane conductance regulator (CFTR)-targeting drug ivacaftor results in improved pulmonary function. "
05/01/2009 - "The sweat test remains the gold standard for the diagnosis of Cystic Fibrosis (CF) even despite the availability of molecular analysis of Cystic Fibrosis Transmembrane Conductance Regulator gene (CFTR). "
|10.||Mannitol (Osmitrol)FDA LinkGeneric
07/09/2012 - "A sustained, significant (p<0.001) improvement in forced expiratory volume in 1 second (FEV(1)) [mean absolute change from baseline over 26 weeks; primary endpoint] was seen in patients with cystic fibrosis who received inhaled mannitol, compared with the control group, in the CF-301 trial, but not in the CF-302 trial (p=0.059). "
03/01/2015 - "Inhaled mannitol is a safe and effective option in adult patients with cystic fibrosis.Mannitol"
01/01/2015 - " clinical implications from this review suggest that mannitol could be considered as a treatment in cystic fibrosis; however, studies comparing its efficacy against other (established) mucolytic therapies need to be undertaken before it can be considered for mainstream practice."
01/01/2015 - "There is evidence to show that treatment with mannitol over a six-month period is associated with an improvement in some measures of lung function in people with cystic fibrosis compared to control. "
01/01/2015 - "There was an initial test to see if participants tolerated mannitol, with only those who could tolerate the drug being randomised to the studies; therefore the study results are not applicable to the cystic fibrosis population as a whole.For"
01/01/2014 - "Despite its complexity, lung transplantation in the cystic fibrosis population is safe, with good outcomes if the clinician is aware of all the potential pathogens and remains vigilant by means of surveillance and proactive treatment. "
12/01/2011 - "The question addressed was whether lung transplantation remained a beneficial treatment for cystic fibrosis (CF) patients infected or colonized with Burkholderia cepacia complex (BCC) prior to lung transplantation (LTx). "
12/01/1997 - "End-stage cystic fibrosis: improved diabetes control 2 years after successful isolated pancreatic cell and double-lung transplantation."
05/01/1992 - "These data support the therapeutic efficacy of lung transplantation for patients with cystic fibrosis."
10/01/2008 - "Moreover, there are several series describing long-term outcomes and demonstrating improved long-term survival following lung transplantation for patients with cystic fibrosis. "
06/01/2000 - "To compare patients' preference and the efficacy of two enteric coated microsphere preparations in patients with cystic fibrosis. "
01/01/1990 - "A comparative study of the efficacy of pH sensitive enteric coated microspheres (ECM) with an enteric coated tablet (ECT) pancreatic enzyme preparation was carried out in 20 children with cystic fibrosis in a double-blind double-placebo crossover manner. "
11/01/2004 - "Microsphere bead arrays and sequence validation of 5/7/9T genotypes for multiplex screening of cystic fibrosis polymorphisms."
06/01/2000 - "Comparison of two enteric coated microsphere preparations in the treatment of pancreatic exocrine insufficiency caused by cystic fibrosis."
05/01/2000 - "Microspheres may be used in gene therapy for pulmonary affections, in particular cystic fibrosis."
10/01/2009 - "The impact of Bcc colonization in cystic fibrosis patients on outcome has been nuanced; thus, cystic fibrosis lung transplant candidates colonized by particular Bcc strains may be transplanted with good outcomes. "
08/01/2015 - "Data were derived from the INSPIRE trial, a dual-site randomized controlled trial of coping skills training in 389 lung transplant candidates (obstructive [48.3%], restrictive [24.2%], cystic fibrosis [13.6%], and other [13.9%]). "
01/01/2015 - "Implementation of a cystic fibrosis lung transplant referral patient decision aid in routine clinical practice: an observational study."
06/01/2014 - "There has been little study on the variability of CsA pharmacokinetics in stable lung transplant (LT) recipients without cystic fibrosis. "
08/01/2010 - "The study included 57 unique and consecutive adult patients (33 women) with cystic fibrosis who underwent lung transplant evaluation at the University of Florida. "
|4.||Drug Therapy (Chemotherapy)
01/11/2002 - "Thus, these results support that P18 may prove to be a salt-resistant antibiotic peptide potentially useful in the treatment of cystic fibrosis patients as well as a valuable adjuvant for antimicrobial chemotherapy."
12/01/2004 - "Clinical improvement in cystic fibrosis following anti-tumourous chemotherapy."
08/01/1981 - "A double-blind controlled trial of anti-Pseudomonas chemotherapy of acute respiratory exacerbations in patients with cystic fibrosis."
10/01/2015 - "Ultimately, we need a personalized human model system for cystic fibrosis with the capacity for uncomplicated bankability, widespread availability, and universal applicability for patient-specific disease modeling, novel pharmacotherapy investigation and screening, and readily executable genetic modification."
01/01/2015 - "Personalized drug therapy for cystic fibrosis (CF) is a long-term dream for CF patients, caregivers, physicians and researchers. "
|5.||Nebulizers and Vaporizers (Inhaler)
04/01/2010 - "This exploratory study assessed the acceptability of slow, deep inhalations in 20 patients with cystic fibrosis (CF) during up to eight simulated nebulizer treatments with the I-neb AAD System. "
04/01/2010 - "We conducted a 6-week open study in nineteen patients with cystic fibrosis enrolled from three centers, to investigate patient perception of a telehealth enabled nebulizer system (Prodose Adaptive Aerosol Delivery [AAD] System), which enabled the doorstep delivery of repeat medication. "
12/01/2007 - "Depending on the studies, contamination of the nebulizers may reach up to 55% of the devices in patients with cystic fibrosis. "
12/01/2004 - "Nebulizer use and maintenance by cystic fibrosis patients: a survey study."
01/01/2001 - "The objective of this study was to evaluate relative efficiency in vitro of four reusable breath-enhanced nebulizers (Pari LC Star, Medic-Aid Ventstream, Devilbiss PermaNeb, Salter Ultramist), and to integrate the in vitro performance data of the nebulizers with the respiratory patterns of four cystic fibrosis (CF) patients to compare efficiency in vivo of each device for each individual patient. "