Current and future treatment options for cystic fibrosis lung disease: latest evidence and clinical implications

Novel Approaches for Potential Therapy of Cystic Fibrosis

(2015) Victoria Sawczak et al.   CURRENT DRUG TARGETS

Reduced risk of nontuberculous mycobacteria in cystic fibrosis adults receiving long-term azithromycin

(2015) Nathalie Coolen et al.   Journal of Cystic Fibrosis

A phase 3, open-label, randomized trial to evaluate the safety and efficacy of levofloxacin inhalation solution (APT-1026) versus tobramycin inhalation solution in stable cystic fibrosis patients

(2015) J. Stuart Elborn et al.   Journal of Cystic Fibrosis

Long-term treatment with oral N-acetylcysteine: Affects lung function but not sputum inflammation in cystic fibrosis subjects. A phase II randomized placebo-controlled trial

(2015) C. Conrad et al.   Journal of Cystic Fibrosis

Early Life Growth Trajectories in Cystic Fibrosis are Associated with Pulmonary Function at Age 6 Years

(2015) Don B. Sanders et al.   JOURNAL OF PEDIATRICS

Voriconazole phototoxicity in children: A retrospective review

(2015) Johanna Sheu et al.   JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY

Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR

(2015) Claire E. Wainwright et al.   NEW ENGLAND JOURNAL OF MEDICINE

Lung Transplantation for Cystic Fibrosis: Results, Indications, Complications, and Controversies

(2015) David Sayah et al.   SEMINARS IN RESPIRATORY AND CRITICAL CARE MEDICINE

Nontuberculous Mycobacteria in Cystic Fibrosis and Non–Cystic Fibrosis Bronchiectasis

(2015) In Park et al.   SEMINARS IN RESPIRATORY AND CRITICAL CARE MEDICINE

Repeated nebulisation of non-viral CFTR gene therapy in patients with cystic fibrosis: a randomised, double-blind, placebo-controlled, phase 2b trial

(2015) Eric W F W Alton et al.   Lancet Respiratory Medicine

Efficacy and safety of ivacaftor in patients with cystic fibrosis who have an Arg117His-CFTR mutation: a double-blind, randomised controlled trial

(2015) Richard B Moss et al.   Lancet Respiratory Medicine

Gene therapy in cystic fibrosis

(2014) D. K. Armstrong et al.   ARCHIVES OF DISEASE IN CHILDHOOD

Antifungal treatment in allergic bronchopulmonary aspergillosis with and without cystic fibrosis: a systematic review

(2014) A. S. Moreira et al.   CLINICAL AND EXPERIMENTAL ALLERGY

Nebulized liposomal amikacin for the treatment ofPseudomonas aeruginosainfection in cystic fibrosis patients

(2014) Zarmina Ehsan et al.   EXPERT OPINION ON INVESTIGATIONAL DRUGS

Colistimethate sodium powder and tobramycin powder for inhalation for the treatment of chronic Pseudomonas aeruginosa lung infection in cystic fibrosis: systematic review and economic model

(2014) P Tappenden et al.   HEALTH TECHNOLOGY ASSESSMENT

A randomized double blind, placebo controlled phase 2 trial of BIIL 284 BS (an LTB4 receptor antagonist) for the treatment of lung disease in children and adults with cystic fibrosis

(2014) M.W. Konstan et al.   Journal of Cystic Fibrosis

Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation

(2014) Kris De Boeck et al.   Journal of Cystic Fibrosis

Inhaled versus nebulised tobramycin: A real world comparison in adult cystic fibrosis (CF)

(2014) M.J. Harrison et al.   Journal of Cystic Fibrosis

Potentiator ivacaftor abrogates pharmacological correction of  F508 CFTR in cystic fibrosis

(2014) D. M. Cholon et al.   Science Translational Medicine

Respiratory microbiota: addressing clinical questions, informing clinical practice

(2014) Geraint B Rogers et al.   THORAX

Ataluren for the treatment of nonsense-mutation cystic fibrosis: a randomised, double-blind, placebo-controlled phase 3 trial

(2014) Eitan Kerem et al.   Lancet Respiratory Medicine

Omalizumab: a new treatment option for allergic bronchopulmonary aspergillosis in patients with cystic fibrosis

(2014) Sylvia Lehmann et al.   Therapeutic Advances in Respiratory Disease

Outcomes and Treatment of Chronic Methicillin-ResistantStaphylococcus aureusDiffers by Staphylococcal Cassette Chromosomemec(SCCmec) Type in Children With Cystic Fibrosis

(2014) Sonya L. Heltshe et al.   Journal of the Pediatric Infectious Diseases Society

Efficacy and Safety of Ivacaftor in Patients Aged 6 to 11 Years with Cystic Fibrosis with aG551DMutation

(2013) Jane C. Davies et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Cystic Fibrosis Pulmonary Guidelines

(2013) Peter J. Mogayzel et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Phase 3 Randomized Study of the Efficacy and Safety of Inhaled Dry Powder Mannitol for the Symptomatic Treatment of Non-Cystic Fibrosis Bronchiectasis

(2013) Diana Bilton et al.   CHEST

Hyaluronic Acid Improves the Tolerability of Hypertonic Saline in the Chronic Treatment of Cystic Fibrosis Patients: A Multicenter, Randomized, Controlled Clinical Trial

(2013) Mirco Ros et al.   Journal of Aerosol Medicine and Pulmonary Drug Delivery

Novel immunologic classification of aspergillosis in adult cystic fibrosis

(2013) Caroline G. Baxter et al.   JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

Inhaled aztreonam for chronic Burkholderia infection in cystic fibrosis: A placebo-controlled trial

(2013) D. Elizabeth Tullis et al.   Journal of Cystic Fibrosis

Whole-genome sequencing to identify transmission of Mycobacterium abscessus between patients with cystic fibrosis: a retrospective cohort study

(2013) Josephine M Bryant et al.   LANCET

Omalizumab therapy for allergic bronchopulmonary aspergillosis in children with cystic fibrosis: A synthesis of published evidence

(2013) Kalliopi Tanou et al.   PEDIATRIC PULMONOLOGY

High-dose ibuprofen is not associated with increased biomarkers of kidney injury in patients with cystic fibrosis

(2013) Thomas Lahiri et al.   PEDIATRIC PULMONOLOGY

Long-term multicentre randomised controlled study of high frequency chest wall oscillation versus positive expiratory pressure mask in cystic fibrosis

(2013) Maggie Patricia McIlwaine et al.   THORAX

Phase II studies of nebulised Arikace in CF patients withPseudomonas aeruginosainfection

(2013) J P Clancy et al.   THORAX

A new era in the treatment of cystic fibrosis: correction of the underlying CFTR defect

(2013) Michael P Boyle et al.   Lancet Respiratory Medicine

Management of comorbidities in older patients with cystic fibrosis

(2013) Barry J Plant et al.   Lancet Respiratory Medicine

Assessment of F/HN-Pseudotyped Lentivirus as a Clinically Relevant Vector for Lung Gene Therapy

(2012) Uta Griesenbach et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Ivacaftor in Subjects With Cystic Fibrosis Who Are Homozygous for the F508del-CFTR Mutation

(2012) Patrick A. Flume et al.   CHEST

Inhaled Hypertonic Saline in Infants and Children Younger Than 6 Years With Cystic Fibrosis

(2012) Margaret Rosenfeld et al.   JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION

Long term effects of denufosol tetrasodium in patients with cystic fibrosis

(2012) Felix Ratjen et al.   Journal of Cystic Fibrosis

Inhaled aztreonam lysine vs. inhaled tobramycin in cystic fibrosis: A comparative efficacy trial

(2012) Baroukh M. Assael et al.   Journal of Cystic Fibrosis

Inhaled antibiotics in cystic fibrosis: what's new?

(2012) Simon Langton Hewer   JOURNAL OF THE ROYAL SOCIETY OF MEDICINE

Safety, efficacy and convenience of colistimethate sodium dry powder for inhalation (Colobreathe DPI) in patients with cystic fibrosis: a randomised study

(2012) Antje Schuster et al.   THORAX

Nebulized hypertonic saline containing hyaluronic acid improves tolerability in patients with cystic fibrosis and lung disease compared with nebulized hypertonic saline alone: a prospective, randomized, double-blind, controlled study

(2012) Maria Lucia Furnari et al.   Therapeutic Advances in Respiratory Disease

Long-Term Inhaled Dry Powder Mannitol in Cystic Fibrosis

(2011) Moira L. Aitken et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Fosfomycin/Tobramycin for Inhalation in Patients with Cystic Fibrosis withPseudomonasAirway Infection

(2011) Bruce C. Trapnell et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Inhaled dry powder mannitol in cystic fibrosis: an efficacy and safety study

(2011) D. Bilton et al.   EUROPEAN RESPIRATORY JOURNAL

Azithromycin blocks autophagy and may predispose cystic fibrosis patients to mycobacterial infection

(2011) Maurizio Renna et al.   JOURNAL OF CLINICAL INVESTIGATION

Aztreonam for inhalation solution (AZLI) in patients with cystic fibrosis, mild lung impairment, and P. aeruginosa

(2011) C.E. Wainwright et al.   Journal of Cystic Fibrosis

Effect of dornase alfa on inflammation and lung function: Potential role in the early treatment of cystic fibrosis

(2011) Michael W. Konstan et al.   Journal of Cystic Fibrosis

A CFTR Potentiator in Patients with Cystic Fibrosis and theG551DMutation

(2011) Bonnie W. Ramsey et al.   NEW ENGLAND JOURNAL OF MEDICINE

Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for theF508del-CFTRmutation

(2011) J P Clancy et al.   THORAX

Hyaluronic acid improves “pleasantness” and tolerability of nebulized hypertonic saline in a cohort of patients with cystic fibrosis

(2010) Paolo Buonpensiero et al.   ADVANCES IN THERAPY

Denufosol Tetrasodium in Patients with Cystic Fibrosis and Normal to Mildly Impaired Lung Function

(2010) Frank J. Accurso et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

The effect of dornase alfa on ventilation inhomogeneity in patients with cystic fibrosis

(2010) R. Amin et al.   EUROPEAN RESPIRATORY JOURNAL

Safety, efficacy and convenience of tobramycin inhalation powder in cystic fibrosis patients: The EAGER trial

(2010) Michael W. Konstan et al.   Journal of Cystic Fibrosis

An 18-month study of the safety and efficacy of repeated courses of inhaled aztreonam lysine in cystic fibrosis

(2010) Christopher M. Oermann et al.   PEDIATRIC PULMONOLOGY

Nutritional considerations in patients with cystic fibrosis

(2010) Anne Munck   Expert Review of Respiratory Medicine

Intravenous monthly pulse methylprednisolone treatment for ABPA in patients with cystic fibrosis

(2009) Malena Cohen-Cymberknoh et al.   Journal of Cystic Fibrosis