相关参考文献
注意：仅列出部分参考文献，下载原文获取全部文献信息。Ivacaftor in Infants Aged 4 to <12 Months with Cystic Fibrosis and a Gating Mutation Results of a Two-Part Phase 3 Clinical Trial
Jane C. Davies et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2021)
Real-World Safety of CFTR Modulators in the Treatment of Cystic Fibrosis: A Systematic Review
Renee V. E. Dagenais et al.
JOURNAL OF CLINICAL MEDICINE (2021)
Disease progression in patients with cystic fibrosis treated with ivacaftor: Data from national US and UK registries
Nataliya Volkova et al.
JOURNAL OF CYSTIC FIBROSIS (2020)
Ivacaftor in cystic fibrosis with residual function: Lung function results from an N-of-1 study
Jerry A. Nick et al.
JOURNAL OF CYSTIC FIBROSIS (2020)
Evaluation of a five-year predicted survival model for cystic fibrosis in later time periods
Theodore G. Liou et al.
SCIENTIFIC REPORTS (2020)
Impact of CFTR modulator use on outcomes in people with severe cystic fibrosis lung disease
Michal Shteinberg et al.
EUROPEAN RESPIRATORY REVIEW (2020)
From Ivacaftor to Triple Combination: A Systematic Review of Efficacy and Safety of CFTR Modulators in People with Cystic Fibrosis
Andrea Gramegna et al.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2020)
Effectiveness of ivacaftor in cystic fibrosis patients with non-G551D gating mutations
Jennifer Guimbellot et al.
JOURNAL OF CYSTIC FIBROSIS (2019)
IVACAFTOR restores FGF19 regulated bile acid homeostasis in cystic fibrosis patients with an S1251N or a G551D gating mutation
Ivo P. van de Peppel et al.
JOURNAL OF CYSTIC FIBROSIS (2019)
P253 Use of ivacaftor (IVA) in patients heterozygous for R117H mutation: real-life experience in a large UK adult CF centre
G. Spoletini et al.
Journal of Cystic Fibrosis (2019)
WS06-4 Ivacaftor (IVA) treatment in patients 6 to
J. Davies et al.
Journal of Cystic Fibrosis (2019)
P256 The effect of ivacaftor on adult cystic fibrosis patients at the Royal Hospital in Oman
Z. Al-Rashdi et al.
Journal of Cystic Fibrosis (2019)
P252 Real world ivacaftor efficacy in children: five years on …
E.A. Robson et al.
Journal of Cystic Fibrosis (2019)
P254 Ivacaftor treatment in patients with severe lung disease carrying CFTR mutations with residual function
D. Salvatore et al.
Journal of Cystic Fibrosis (2019)
Effectivenesss of ivacaftor in severe cystic fibrosis patients and non-G551D gating mutations
Donatello Salvatore et al.
PEDIATRIC PULMONOLOGY (2019)
Patient-reported outcomes in patients with cystic fibrosis with a G551D mutation on ivacaftor treatment: results from a cross-sectional study
Scott C. Bell et al.
BMC PULMONARY MEDICINE (2019)
Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial
Harry G. M. Heijerman et al.
LANCET (2019)
Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele
P. G. Middleton et al.
NEW ENGLAND JOURNAL OF MEDICINE (2019)
Ivacaftor Is Associated with Reduced Lung Infection by Key Cystic Fibrosis Pathogens A Cohort Study Using National Registry Data
Freddy J. Frost et al.
ANNALS OF THE AMERICAN THORACIC SOCIETY (2019)
Longitudinal Trends in Real-World Outcomes after Initiation of Ivacaftor A Cohort Study from the Cystic Fibrosis Registry of Ireland
Laura Kirwan et al.
ANNALS OF THE AMERICAN THORACIC SOCIETY (2019)
THE EFFECTS OF IVACAFTOR ON PANCREATIC FUNCTION IN PAEDIATRIC PATIENTS WITH CYSTIC FIBROSIS GATING MUTATIONS
Joshua Emery et al.
ARCHIVES OF DISEASE IN CHILDHOOD (2019)
Pseudomonas aeruginosa in cystic fibrosis patients with c.1652G⟩A (G551D)-CFTR treated with ivacaftorChanges in microbiological parameters
B. C. Millar et al.
JOURNAL OF CLINICAL PHARMACY AND THERAPEUTICS (2018)
CORK Study in Cystic Fibrosis Sustained Improvements in Ultra-Low-Dose Chest CT Scores After CFTR Modulation With Ivacaftor
Nicola J. Ronan et al.
CHEST (2018)
Precision Medicine In Action: The Impact Of Ivacaftor On Cystic Fibrosis-Related Hospitalizations
Lisa B. Feng et al.
HEALTH AFFAIRS (2018)
Retrospective observational study of French patients with cystic fibrosis and a G1y551Asp-CFTR mutation after 1 and 2 years of treatment with ivacaftor in a real-world setting
Dominique Hubert et al.
JOURNAL OF CYSTIC FIBROSIS (2018)
WS05.4 Clinical effectiveness results from the first interim analysis of the VOCAL study; an observational study of ivacaftor in patients with cystic fibrosis and selected non-G551D gating mutations
C. Castellani et al.
Journal of Cystic Fibrosis (2018)
IPD2.05 Effects of ivacaftor in patients with cystic fibrosis and severe lung disease carrying CFTR mutations with residual function
D. Salvatore et al.
Journal of Cystic Fibrosis (2018)
IPD2.03 The effects of 3-year ivacaftor use on lung function and intravenous days seen in UK CF Registry Data
S.J. Newsome et al.
Journal of Cystic Fibrosis (2018)
IPD2.04 Long-term effects of ivacaftor in patients with G551D mutation and mild lung disease
H. Ellemunter et al.
Journal of Cystic Fibrosis (2018)
IPD2.06 Ivacaftor therapy in patients with severe baseline lung disease carrying a residual function mutation
R.M. Mitchell et al.
Journal of Cystic Fibrosis (2018)
P204 An increase in weight and fat mass observed following five months of ivacaftor treatment plateaus at 24 months in adults with G551D-related cystic fibrosis
A. Tierney et al.
Journal of Cystic Fibrosis (2018)
Reduction of Recurrence Risk of Pancreatitis in Cystic Fibrosis With Ivacaftor: Case Series
Andres Carrion et al.
JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION (2018)
Energy Balance and Mechanisms of Weight Gain with Ivacaftor Treatment of Cystic Fibrosis Gating Mutations
Virginia A. Stallings et al.
JOURNAL OF PEDIATRICS (2018)
Data from the US and UK cystic fibrosis registries support disease modification by CFTR modulation with ivacaftor
Leona Bessonova et al.
THORAX (2018)
AN OBSERVATIONAL STUDY OF IVACAFTOR IN PATIENTS WITH CYSTIC FIBROSIS AND SELECTED NON-G551D GATING MUTATIONS IN UK, ITALY AND NETHERLANDS: HEALTHCARE RESOURCE UTILIZATION FROM THE FIRST INTERIM ANALYSIS OF THE VOCAL STUDY
C. Castellani et al.
VALUE IN HEALTH (2018)
Ivacaftor treatment of cystic fibrosis in children aged 12 to <24 months and with a CFTR gating mutation (ARRIVAL): a phase 3 single-arm study
Margaret Rosenfeld et al.
LANCET RESPIRATORY MEDICINE (2018)
SLC26A9 Gene Is Associated With Lung Function Response to Ivacaftor in Patients With Cystic Fibrosis
Harriet Corvol et al.
FRONTIERS IN PHARMACOLOGY (2018)
AN OBSERVATIONAL STUDY OF IVACAFTOR IN PATIENTS WITH CYSTIC FIBROSIS IN FRANCE: FIRST INTERIM ANALYSIS OF HEALTH CARE RESOURCE UTILIZATION FROM THE BRIO STUDY
D. Hubert et al.
VALUE IN HEALTH (2018)
The burden of cystic fibrosis in the Medicaid population
Mariam Hassan et al.
CLINICOECONOMICS AND OUTCOMES RESEARCH (2018)
Environmental scan of cystic fibrosis research worldwide
Janice Kelly
JOURNAL OF CYSTIC FIBROSIS (2017)
Ivacaftor for the p.Ser549Arg (S549R) gating mutation - The Israeli experience
Adi Dagan et al.
RESPIRATORY MEDICINE (2017)
Keep them breathing: Cystic fibrosis pathophysiology, diagnosis, and treatment
Sheena D. Brown et al.
JAAPA-JOURNAL OF THE AMERICAN ACADEMY OF PHYSICIAN ASSISTANTS (2017)
Patients with Cystic Fibrosis and a G551D or Homozygous F508del Mutation: Similar Lung Function Decline
Gregory S. Sawicki et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2017)
Restoring Cystic Fibrosis Transmembrane Conductance Regulator Function Reduces Airway Bacteria and Inflammation in People with Cystic Fibrosis and Chronic Lung Infections
Katherine B. Hisert et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2017)
Long-term computed tomographic changes in cystic fibrosis patients treated with ivacaftor
Guillaume Chassagnon et al.
EUROPEAN RESPIRATORY JOURNAL (2016)
Bone demineralization is improved by ivacaftor in patients with cystic fibrosis carrying the p.Gly551Asp mutation
Isabelle Sermet-Gaudelus et al.
Journal of Cystic Fibrosis (2016)
263 Comparison of FEV1 and BMI of the 4 common CF mutations in UAE
K.Y. Al Redha et al.
Journal of Cystic Fibrosis (2016)
31 Effects of ivacaftor in cystic fibrosis patients carrying a non-G551D gating mutation
P. Iacotucci et al.
Journal of Cystic Fibrosis (2016)
28 Reduction in pulmonary exacerbations (PEx) after initiation of ivacaftor: a retrospective cohort study among patients with cystic fibrosis (CF) treated in real-world settings
M. Hassan et al.
Journal of Cystic Fibrosis (2016)
Lung clearance index response in patients with CF with class III CFTR mutations
Mica Kane et al.
THORAX (2016)
Healthcare resource utilization associated with ivacaftor use in patients with cystic fibrosis
Ellison D. Suthoff et al.
JOURNAL OF MEDICAL ECONOMICS (2016)
Safety, pharmacokinetics, and pharmacodynamics of ivacaftor in patients aged 2-5 years with cystic fibrosis and a CFTR gating mutation (KIWI): an open-label, single-arm study
Jane C. Davies et al.
LANCET RESPIRATORY MEDICINE (2016)
Ivacaftor-as effective in clinical practice?
Ayesha Aziz et al.
EUROPEAN RESPIRATORY JOURNAL (2016)
Intestinal Current Measurements Detect Activation of Mutant CFTR in Patients with Cystic Fibrosis with the G551D Mutation Treated with Ivacaftor
Simon Y. Graeber et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2015)
Sustained Benefit from Ivacaftor Demonstrated by Combining Clinical Trial and Cystic Fibrosis Patient Registry Data
Gregory S. Sawicki et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2015)
Ivacaftor improves appearance of sinus disease on computerised tomography in cystic fibrosis patients with G551D mutation
S. I. Sheikh et al.
CLINICAL OTOLARYNGOLOGY (2015)
Effect of ivacaftor therapy on exhaled nitric oxide in patients with cystic fibrosis
Hartmut Grasemann et al.
JOURNAL OF CYSTIC FIBROSIS (2015)
Computed tomography correlates with improvement with ivacaftor in cystic fibrosis patients with G551D mutation
Shahid I. Sheikh et al.
JOURNAL OF CYSTIC FIBROSIS (2015)
128 The metabolic consequences of CFTR modulation with ivacaftor in a single adult cystic fibrosis centre cohort
G. Ronan et al.
Journal of Cystic Fibrosis (2015)
WS16.1 Clinical Outcomes of Real-World Kalydeco (CORK) study – Investigating the impact of CFTR potentiation on the intestinal microbiota, exocrine pancreatic function and intestinal inflammation prospectively over 12 months
J. Deane et al.
Journal of Cystic Fibrosis (2015)
135 Transformational care at the All Wales Adult CF Centre (AWACFC) – the impact of ivacaftor (Kalydeco®) one year on
L. Guhaniyogi et al.
Journal of Cystic Fibrosis (2015)
182 Impact of ivacaftor on glycaemic health in patients carrying the G551D mutation
P.J. Barry et al.
Journal of Cystic Fibrosis (2015)
Clinical Updates in Cystic Fibrosis-Related Diabetes
Amanda L. Brennan et al.
SEMINARS IN RESPIRATORY AND CRITICAL CARE MEDICINE (2015)
Pseudomonas aeruginosa in Cystic Fibrosis Patients With G551D-CFTR Treated With Ivacaftor
Sonya L. Heltshe et al.
CLINICAL INFECTIOUS DISEASES (2015)
Clinical Mechanism of the Cystic Fibrosis Transmembrane Conductance Regulator Potentiator Ivacaftor in G551D-mediated Cystic Fibrosis
Steven M. Rowe et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2014)
Effects of Ivacaftor in Patients With Cystic Fibrosis Who Carry the G551D Mutation and Have Severe Lung Disease
Peter J. Barry et al.
CHEST (2014)
Ivacaftor for the treatment of patients with cystic fibrosis and the G551D mutation: a systematic review and cost-effectiveness analysis
Penny Whiting et al.
HEALTH TECHNOLOGY ASSESSMENT (2014)
WS20.6 The use of LCI as an effective tool for monitoring clinical response to ivacaftor therapy in CF patients with at least one G551D-allele
L.E. Jenkins et al.
Journal of Cystic Fibrosis (2014)
Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation
Kris De Boeck et al.
JOURNAL OF CYSTIC FIBROSIS (2014)
HOME OXYGEN THERAPY ASSESSMENT FOR COPD PATIENTS DISCHARGED FROM HOSPITAL: RESPIRATORY NP MODEL OF CARE-A PILOT STUDY
T. Hall
RESPIROLOGY (2014)
Sweat chloride is not a useful marker of clinical response to Ivacaftor
Peter J. Barry et al.
THORAX (2014)
THE EFFECT OF IVACAFTOR THERAPY ON THE MICROBIAL DIVERSITY OF CYSTIC FIBROSIS LUNG INFECTION
H. D. Green et al.
THORAX (2014)
PROSPECTIVE EXAMINATION OF THE EFFECTS OF IVACAFTOR ON GLYCAEMIC HEALTH
A. Banerjee et al.
THORAX (2014)
Long-term safety and efficacy of ivacaftor in patients with cystic fibrosis who have the Gly551Asp-CFTR mutation: a phase 3, open-label extension study (PERSIST)
Edward F. McKone et al.
LANCET RESPIRATORY MEDICINE (2014)
Efficacy and Safety of Ivacaftor in Patients Aged 6 to 11 Years with Cystic Fibrosis with a G551D Mutation
Jane C. Davies et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2013)
Effects of ivacaftor on severely ill patients with cystic fibrosis carrying a G551D mutation
Helge Hebestreit et al.
JOURNAL OF CYSTIC FIBROSIS (2013)
DOES IVACAFTOR IMPROVE OBJECTIVE MEASUREMENTS OF HEALTH IN PATIENTS WITH THE G551D CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR (CFTR) PROTEIN MUTATION? THE EXPERIENCE OF A UK CYSTIC FIBROSIS CENTRE
A. E. Ewence et al.
THORAX (2013)
42nd ESCP symposium on clinical pharmacy: implementation of pharmacy practice; Prague, Czech Republic, 16-18 October 2013
International Journal of Clinical Pharmacy (2013)
Psychometric evaluation of the Cystic Fibrosis Questionnaire-Revised in a national sample
Alexandra L. Quittner et al.
QUALITY OF LIFE RESEARCH (2012)
A CFTR Potentiator in Patients with Cystic Fibrosis and the G551D Mutation
Bonnie W. Ramsey et al.
NEW ENGLAND JOURNAL OF MEDICINE (2011)
Failure to Recover to Baseline Pulmonary Function after Cystic Fibrosis Pulmonary Exacerbation
Don B. Sanders et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2010)
Year-to-year changes in lung function in individuals with cystic fibrosis
Theodore G. Liou et al.
JOURNAL OF CYSTIC FIBROSIS (2010)
Preferred Reporting Items for Systematic Reviews and Meta-Analyses: The PRISMA Statement
David Moher et al.
PLOS MEDICINE (2009)
Longitudinal changes in growth parameters are correlated with changes in pulmonary function in children with cystic fibrosis
ML Peterson et al.
PEDIATRICS (2003)
Effect of genotype on phenotype and mortality in cystic fibrosis: a retrospective cohort study
EF McKone et al.
LANCET (2003)
Psychometric and clinimetric validity of the 20-Item Sino-Nasal Outcome Test (SNOT-20)
JF Piccirillo et al.
OTOLARYNGOLOGY-HEAD AND NECK SURGERY (2002)
Predictive 5-year survivorship model of cystic fibrosis
TG Liou et al.
AMERICAN JOURNAL OF EPIDEMIOLOGY (2001)
Longitudinal relationship among growth, nutritional status, and pulmonary function in children with cystic fibrosis: Analysis of the Cystic Fibrosis Foundation National CF Patient registry
BS Zemel et al.
JOURNAL OF PEDIATRICS (2000)
分享论文
