Real-World Safety of CFTR Modulators in the Treatment of Cystic Fibrosis: A Systematic Review

Outcomes of pregnancy in women with cystic fibrosis (CF) taking CFTR modulators – an international survey

(2020) Edward F Nash et al.   Journal of Cystic Fibrosis

Testicular pain following initiation of elexacaftor/tezacaftor/ivacaftor in males with cystic fibrosis

(2020) Shannon M. Rotolo et al.   Journal of Cystic Fibrosis

Impact of CFTR modulator use on outcomes in people with severe cystic fibrosis lung disease

(2020) Michal Shteinberg et al.   European Respiratory Review

Drug allergy to CFTR modulator therapy associated with lumacaftor-specific CD4+ T lymphocytes

(2020) Jobst F. Roehmel et al.   JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

Tezacaftor/ivacaftor in people with cystic fibrosis who stopped lumacaftor/ivacaftor due to respiratory adverse events

(2020) Carsten Schwarz et al.   Journal of Cystic Fibrosis

Biliary disease and cholecystectomy after initiation of elexacaftor/ivacaftor/tezacaftor in adults with cystic fibrosis

(2020) Julie Safirstein et al.   Journal of Cystic Fibrosis

Clinical response to lumacaftor-ivacaftor in patients with cystic fibrosis according to baseline lung function

(2020) Pierre-Régis Burgel et al.   Journal of Cystic Fibrosis

Azithromycin and tezacaftor/ivacaftor is associated with first-degree heart block in an adult with cystic fibrosis

(2020) Yang Song et al.   Journal of Cystic Fibrosis

Decreased survival in cystic fibrosis patients with a positive screen for depression

(2020) Michael S. Schechter et al.   Journal of Cystic Fibrosis

Trikafta and Psychopathology in Cystic Fibrosis: A Case Report

(2020) William Tindell et al.   PSYCHOSOMATICS

Safety and Efficacy of Elexacaftor/Tezacaftor/Ivacaftor for ≥24 Weeks in People With CF and ≥1 F508del Allele: Interim Results of an Open-Label Phase Three Clinical Trial

(2020) Matthias Griese et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

From Ivacaftor to Triple Combination: A Systematic Review of Efficacy and Safety of CFTR Modulators in People with Cystic Fibrosis

(2020) Andrea Gramegna et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Ivacaftor drug desensitization

(2019) Alexander Patterson et al.   PEDIATRIC PULMONOLOGY

Use of ivacaftor in late diagnosed cystic fibrosis monozygotic twins heterozygous for F508del and R117H-7T – a case report

(2019) Matthias Welsner et al.   BMC Pulmonary Medicine

A Systematic Review of the Clinical Efficacy and Safety of CFTR Modulators in Cystic Fibrosis

(2019) Al-Rahim R. Habib et al.   Scientific Reports

Lumacaftor/ Ivacaftor improves exercise tolerance in patients with Cystic Fibrosis and severe airflow obstruction

(2019) Peter A. B. Wark et al.   BMC Pulmonary Medicine

Successful Pregnancy of a Patient with Cystic Fibrosis Genotype F508del/ F508del and Progressed Pulmonary Destruction on lumacaftor/ivacaftor

(2019) Jochen G. Mainz et al.   KLINISCHE PADIATRIE

Real-Life Safety and Effectiveness of Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis

(2019) Pierre-Régis Burgel et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Cystic fibrosis related diabetes: Pathophysiology, screening and diagnosis

(2019) Andrea Granados et al.   Journal of Cystic Fibrosis

Longitudinal changes in lung function following initiation of lumacaftor/ivacaftor combination

(2019) Ioanna Loukou et al.   Journal of Cystic Fibrosis

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

(2019) Harry G M Heijerman et al.   LANCET

Elexacaftor–Tezacaftor–Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele

(2019) Peter G. Middleton et al.   NEW ENGLAND JOURNAL OF MEDICINE

Lumacaftor/ivacaftor reduces exacerbations in adults homozygous for Phe508del mutation with severe lung disease

(2019) Koliarne Tong et al.   Journal of Cystic Fibrosis

To be or not to be on CFTR modulators during pregnancy: Risks to be considered

(2019) Sona Vekaria et al.   Journal of Cystic Fibrosis

Compassionate Use of Lumacaftor/Ivacaftor in Cystic Fibrosis: Spanish Experience

(2018) Layla Diab-Cáceres et al.   ARCHIVOS DE BRONCONEUMOLOGIA

Ivacaftor withdrawal syndrome in cystic fibrosis patients with the G551D mutation

(2018) Aaron T. Trimble et al.   Journal of Cystic Fibrosis

Lumacaftor/ivacaftor in patients with cystic fibrosis and advanced lung disease homozygous for F508del-CFTR

(2018) Jennifer L. Taylor-Cousar et al.   Journal of Cystic Fibrosis

Measured fetal and neonatal exposure to Lumacaftor and Ivacaftor during pregnancy and while breastfeeding

(2018) Aaron Trimble et al.   Journal of Cystic Fibrosis

Retrospective observational study of French patients with cystic fibrosis and a Gly551Asp- CFTR mutation after 1 and 2 years of treatment with ivacaftor in a real-world setting

(2018) Dominique Hubert et al.   Journal of Cystic Fibrosis

Energy Balance and Mechanisms of Weight Gain with Ivacaftor Treatment of Cystic Fibrosis Gating Mutations

(2018) Virginia A. Stallings et al.   JOURNAL OF PEDIATRICS

Metabolomic responses to lumacaftor/ivacaftor in cystic fibrosis

(2018) Benjamin T. Kopp et al.   PEDIATRIC PULMONOLOGY

First experience in Switzerland in Phe508del homozygous cystic fibrosis patients with end-stage pulmonary disease enrolled in a lumacaftor-ivacaftor therapy trial – preliminary results

(2018)   SWISS MEDICAL WEEKLY

Compassionate Use of Lumacaftor/Ivacaftor in Cystic Fibrosis: Spanish Experience

(2018) Layla Diab-Cáceres et al.   ARCHIVOS DE BRONCONEUMOLOGIA

Efectividad de ivacaftor en vida real en niños con fibrosis quística y mutación G551D

(2018) David Gomez-Pastrana et al.   ANALES DE PEDIATRIA

VX-445–Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles

(2018) Dominic Keating et al.   NEW ENGLAND JOURNAL OF MEDICINE

Corticosteroid use and increased CXCR2 levels on leukocytes are associated with lumacaftor/ivacaftor discontinuation in cystic fibrosis patients homozygous for the F508del CFTR mutation

(2018) Kerstin Pohl et al.   PLoS One

Effect of bronchodilators in healthy individuals receiving lumacaftor/ivacaftor combination therapy

(2017) Gautham Marigowda et al.   Journal of Cystic Fibrosis

Real-life acute lung function changes after lumacaftor/ivacaftor first administration in pediatric patients with cystic fibrosis

(2017) Aurélie Labaste et al.   Journal of Cystic Fibrosis

Worsening anxiety and depression after initiation of lumacaftor/ivacaftor combination therapy in adolescent females with cystic fibrosis

(2017) Cameron J. McKinzie et al.   Journal of Cystic Fibrosis

Gallbladder and bile duct disease in Cystic Fibrosis

(2017) David N. Assis et al.   Journal of Cystic Fibrosis

Peripheral muscle abnormalities in cystic fibrosis: Etiology, clinical implications and response to therapeutic interventions

(2017) Mathieu Gruet et al.   Journal of Cystic Fibrosis

Immediate effects of lumacaftor/ivacaftor administration on lung function in patients with severe cystic fibrosis lung disease

(2017) Natalia Popowicz et al.   Journal of Cystic Fibrosis

Real-life initiation of lumacaftor/ivacaftor combination in adults with cystic fibrosis homozygous for the Phe508del CFTR mutation and severe lung disease

(2017) Dominique Hubert et al.   Journal of Cystic Fibrosis

Tezacaftor–Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis

(2017) Steven M. Rowe et al.   NEW ENGLAND JOURNAL OF MEDICINE

Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del

(2017) Jennifer L. Taylor-Cousar et al.   NEW ENGLAND JOURNAL OF MEDICINE

Cystic Fibrosis Transmembrane Regulator Modulators: Implications for the Management of Depression and Anxiety in Cystic Fibrosis

(2017) Jaideep S. Talwalkar et al.   PSYCHOSOMATICS

Efficacy and safety of lumacaftor and ivacaftor in patients aged 6–11 years with cystic fibrosis homozygous for F508del-CFTR : a randomised, placebo-controlled phase 3 trial

(2017) Felix Ratjen et al.   Lancet Respiratory Medicine

Assessment of safety and efficacy of long-term treatment with combination lumacaftor and ivacaftor therapy in patients with cystic fibrosis homozygous for the F508del-CFTR mutation (PROGRESS): a phase 3, extension study

(2017) Michael W Konstan et al.   Lancet Respiratory Medicine

The impact of 12 months treatment with ivacaftor on Scottish paediatric patients with cystic fibrosis with the G551D mutation: a review

(2016) Carol Dryden et al.   ARCHIVES OF DISEASE IN CHILDHOOD

Gastrointestinal Disorders in Cystic Fibrosis

(2016) David N. Assis et al.   CLINICS IN CHEST MEDICINE

Effect of ivacaftor in patients with advanced cystic fibrosis and a G551D-CFTR mutation: Safety and efficacy in an expanded access program in the United States

(2016) Jennifer Taylor-Cousar et al.   Journal of Cystic Fibrosis

Cystic fibrosis

(2016) J Stuart Elborn   LANCET

Therapeutic challenges posed by critical drug-drug interactions in cystic fibrosis

(2016) Cameron L. Jordan et al.   PEDIATRIC PULMONOLOGY

Efficacy and safety of lumacaftor/ivacaftor combination therapy in patients with cystic fibrosis homozygous for Phe508del CFTR by pulmonary function subgroup: a pooled analysis

(2016) J Stuart Elborn et al.   Lancet Respiratory Medicine

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

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

Symptoms of depression impact the course of lung function in adolescents and adults with cystic fibrosis

(2015) Astrid Fidika et al.   BMC Pulmonary Medicine

European Cystic Fibrosis Society Standards of Care: Framework for the Cystic Fibrosis Centre

(2014) Steven Conway 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

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)

(2014) Edward F McKone et al.   Lancet Respiratory Medicine

Desensitization in delayed drug hypersensitivity reactions - an EAACI position paper of the Drug Allergy Interest Group

(2013) K. Scherer et al.   ALLERGY

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

Effects of ivacaftor on severely ill patients with cystic fibrosis carrying a G551D mutation

(2013) Helge Hebestreit et al.   Journal of Cystic Fibrosis

Malnutrition in Cystic Fibrosis

(2013) Suzanne Culhane et al.   NUTRITION IN CLINICAL PRACTICE

Assessment of clinical response to ivacaftor with lung clearance index in cystic fibrosis patients with a G551D- CFTR mutation and preserved spirometry: a randomised controlled trial

(2013) Jane Davies et al.   Lancet Respiratory Medicine

Variation of serum creatine kinase (CK) levels and prevalence of persistent hyperCKemia in a Norwegian normal population. The Tromsø Study

(2011) Hallvard Lilleng et al.   NEUROMUSCULAR DISORDERS

A CFTR Potentiator in Patients with Cystic Fibrosis and theG551DMutation

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

Sources of Patients' Knowledge of the Adverse Effects of Psychotropic Medication and the Perceived Influence of Adverse Effects on Compliance Among Service Users Attending Community Mental Health Services

(2009) Vincent I.O. Agyapong et al.   JOURNAL OF CLINICAL PSYCHOPHARMACOLOGY

High treatment burden in adults with cystic fibrosis: Challenges to disease self-management

(2008) Gregory S. Sawicki et al.   Journal of Cystic Fibrosis