Isabelle Sermet-Gaudelus

Use of 16S rRNA gene sequencing for identification of nonfermenting gram-negative bacilli recovered from patients attending a single cystic fibrosis center.

Mycobacterium abscessus and children with cystic fibrosis.

Measurement of immunoglobulin G against Mycobacterial antigen A60 in patients with cystic fibrosis and lung infection due to Mycobacterium abscessus.

Age-related prevalence and distribution of nontuberculous mycobacterial species among patients with cystic fibrosis.

Value of the chlorhexidine decontamination method for recovery of nontuberculous mycobacteria from sputum samples of patients with cystic fibrosis.

Myeloperoxidase promoter polymorphism -463G is associated with more severe clinical expression of cystic fibrosis pulmonary disease.

The CF-CIRC study: a French collaborative study to assess the accuracy of cystic fibrosis diagnosis in neonatal screening.

Low bone mineral density in young children with cystic fibrosis.

[Anti Pseudomonas aeruginosa antibiotic therapy in cystic fibrosis (exclusion of macrolides)].

In vitro prediction of stop-codon suppression by intravenous gentamicin in patients with cystic fibrosis: a pilot study.

[Parvovirus B19: importance of diathesis in the clinical expression of a common infection].

[Recommendations for the management of bone demineralization in cystic fibrosis].

Bacterial contamination in the environment of hospitalised children with cystic fibrosis.

Matrix-assisted laser desorption ionization-time of flight mass spectrometry for identification of nonfermenting gram-negative bacilli isolated from cystic fibrosis patients.

Pain in children and adults with cystic fibrosis: a comparative study.

[Novel therapies for cystic fibrosis in 2009].

[Bone health in cystic fibrosis].

Effect of mutator P. aeruginosa on antibiotic resistance acquisition and respiratory function in cystic fibrosis.

Update on cystic fibrosis-related bone disease: a special focus on children.

An international randomized multicenter comparison of nasal potential difference techniques.

Measurement of nasal potential difference in young children with an equivocal sweat test following newborn screening for cystic fibrosis.

Clinical phenotype and genotype of children with borderline sweat test and abnormal nasal epithelial chloride transport.

Guidelines on the early management of infants diagnosed with cystic fibrosis following newborn screening.

Ataluren (PTC124) induces cystic fibrosis transmembrane conductance regulator protein expression and activity in children with nonsense mutation cystic fibrosis.

[French guidelines for sweat test practice and interpretation for cystic fibrosis neonatal screening].

Genotypic and phenotypic variation in Pseudomonas aeruginosa reveals signatures of secondary infection and mutator activity in certain cystic fibrosis patients with chronic lung infections.

Determinants of refusal of A/H1N1 pandemic vaccination in a high risk population: a qualitative approach.

Ivacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation.

Biosynthesis of cystic fibrosis transmembrane conductance regulator.

[National French guidelines for management of infants with cystic fibrosis].

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

European Cystic Fibrosis Society Standards of Care: Best Practice guidelines.

Factors associated with humoral immune response to pandemic A/H1N1(v) 2009 influenza vaccine in cystic fibrosis.

Central venous thrombosis and thrombophilia in cystic fibrosis: A prospective study.

[Weakening osteopathies, chronic kidney disease, malabsorption, biological anomalies of calium/phosphorus metabolism: appropriate indications for a reasoned reimbursment of serum vitamin D measurement].

Pseudomonas aeruginosa eradicates Staphylococcus aureus by manipulating the host immunity.

Persistent Bordetella bronchiseptica infection in a child with cystic fibrosis: Relationship to bacterial phenotype.

Effect of ivacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation: patient-reported outcomes in the STRIVE randomized, controlled trial.

An unexpected effect of TNF-α on F508del-CFTR maturation and function.

US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis.

US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis: executive summary.

Lessons from a French collaborative case-control study in cystic fibrosis patients during the 2009 A/H1N1 influenza pandemy.

Bioelectrical impedance in young patients with cystic fibrosis: Validation of a specific equation and clinical relevance.

Long-Term Rasamsonia argillacea Complex Species Colonization Revealed by PCR Amplification of Repetitive DNA Sequences in Cystic Fibrosis Patients.

Changes of CFTR functional measurements and clinical improvements in cystic fibrosis patients with non p.Gly551Asp gating mutations treated with ivacaftor.

Analysis of nasal potential in murine cystic fibrosis models.

Bone demineralization is improved by ivacaftor in patients with cystic fibrosis carrying the p.Gly551Asp mutation.

French law: what about a reasoned reimbursement of serum vitamin D assays?

Diagnosis of Cystic Fibrosis in Screened Populations.

Diagnosis of Cystic Fibrosis: Consensus Guidelines from the Cystic Fibrosis Foundation.

[Management of infants whose diagnosis is inconclusive at neonatal screening for cystic fibrosis].

A multiple reader scoring system for Nasal Potential Difference parameters.

Correction of CFTR function in nasal epithelial cells from cystic fibrosis patients predicts improvement of respiratory function by CFTR modulators.

Multifocal fixed drug eruption to ceftazidime in a child with cystic fibrosis.

The suppression of premature termination codons and the repair of splicing mutations in CFTR.

Guidelines for the clinical management and follow-up of infants with inconclusive cystic fibrosis diagnosis through newborn screening.

Cis variants identified in F508del complex alleles modulate CFTR channel rescue by small molecules.

Factors influencing readthrough therapy for frequent cystic fibrosis premature termination codons.

ECFS best practice guidelines: the 2018 revision.

N1303K: Leaving no stone unturned in the search for transformational therapeutics.

Correction to: In vitro prediction of stop-codon suppression by intravenous gentamicin in patients with cystic fibrosis: a pilot study.

Structure-guided combination therapy to potently improve the function of mutant CFTRs.

Might Brushed Nasal Cells Be a Surrogate for CFTR Modulator Clinical Response?

Increased expression of ATP12A proton pump in cystic fibrosis airways.

Predictive factors for lumacaftor/ivacaftor clinical response.

Chronic Staphylococcus aureus Lung Infection Correlates With Proteogenomic and Metabolic Adaptations Leading to an Increased Intracellular Persistence.

Emerging Therapeutic Approaches for Cystic Fibrosis. From Gene Editing to Personalized Medicine.

Mycobacterium bolletii Lung Disease in Cystic Fibrosis.

Airway surface liquid acidification initiates host defense abnormalities in Cystic Fibrosis.

A critical review of definitions used to describe Pseudomonas aeruginosa microbiological status in patients with cystic fibrosis for application in clinical trials.

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

Cystic Fibrosis Diagnosis in Newborns, Children, and Adults.

Cystic fibrosis bone disease treatment: Current knowledge and future directions.

Insights into the variability of nasal potential difference, a biomarker of CFTR activity.

Author Correction: Airway surface liquid acidification initiates host defense abnormalities in Cystic Fibrosis.

CFTR: New insights into structure and function and implications for modulation by small molecules.

Suppressing 'nonsense' in cystic fibrosis.

Impact of COVID-19 on people with cystic fibrosis.

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

Urinary Exosomes of Patients with Cystic Fibrosis Unravel CFTR-Related Renal Disease.

A phase 3, double-blind, parallel-group study to evaluate the efficacy and safety of tezacaftor in combination with ivacaftor in participants 6 through 11 years of age with cystic fibrosis homozygous for F508del or heterozygous for the F508del-CFTR mutation and a residual function mutation.

Updated guidance on the management of children with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID).

Exon identity influences splicing induced by exonic variants and in silico prediction efficacy.

Arterial abnormalities identified in kidneys transplanted into children during the COVID-19 pandemic.

Pharmacokinetic and Pharmacodynamic Optimization of Antibiotic Therapy in Cystic Fibrosis Patients: Current Evidences, Gaps in Knowledge and Future Directions.

Modulators of CFTR. Updates on clinical development and future directions.

"Il faut continuer à poser des questions" patient reported outcome measures in cystic fibrosis: An anthropological perspective.

New insights into structure and function of bis-phosphinic acid derivatives and implications for CFTR modulation.

Prior infection by seasonal coronaviruses, as assessed by serology, does not prevent SARS-CoV-2 infection and disease in children, France, April to June 2020.

Airway Surface Liquid pH Regulation in Airway Epithelium Current Understandings and Gaps in Knowledge.

Involvement of CFTR in the pathogenesis of pulmonary arterial hypertension.

Antisense oligonucleotide-based drug development for Cystic Fibrosis patients carrying the 3849+10 kb C-to-T splicing mutation.

Insights Into Patient Variability During Ivacaftor-Lumacaftor Therapy in Cystic Fibrosis.

Sweat Chloride Testing and Nasal Potential Difference (NPD) Are Primary Outcome Parameters in Treatment with Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulators.

Lumacaftor-ivacaftor effects on cystic fibrosis-related liver involvement in adolescents with homozygous F508 del-CFTR.

Therapeutic pipeline for individuals with cystic fibrosis with mutations nonresponsive to current cystic fibrosis transmembrane conductance regulator modulators.

Author Correction: New insights into structure and function of bis-phosphinic acid derivatives and implications for CFTR modulation.

Pharmacological chaperones improve intra-domain stability and inter-domain assembly via distinct binding sites to rescue misfolded CFTR.

Correlating genotype with phenotype using CFTR-mediated whole-cell Cl currents in human nasal epithelial cells.

Inflammation biomarkers in sputum for clinical trials in cystic fibrosis: current understanding and gaps in knowledge.

Comparison of Transient Elastography, ShearWave Elastography, Magnetic Resonance Elastography and FibroTest as routine diagnostic markers for assessing liver fibrosis in children with Cystic Fibrosis.

Reclassifying inconclusive diagnosis after newborn screening for cystic fibrosis. Moving forward.

A Phase 3, open-label, 96-week trial to study the safety, tolerability, and efficacy of tezacaftor/ivacaftor in children ≥ 6 years of age homozygous for F508del or heterozygous for F508del and a residual function CFTR variant.

Systemic bis-phosphinic acid derivative restores chloride transport in Cystic Fibrosis mice.