Isabelle Sermet-Gaudelus

Isabelle Sermet-Gaudelus

Service de Pneumologie et Allergologie Pédiatriques and Center de Ressources et de Compétence de la Mucoviscidose, Hôpital Necker Enfants Malades

Affiliated withHôpital Necker Enfants MaladesInstitut Necker Enfants MaladesUniversité de paris

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Research Area

Biography

Isabelle Sermet-Gaudelus is a professor in Paediatric at the Necker-Enfants Malades Hospital, Paris France. She graduated from the University Paul Sabatier, Toulouse in 1989 and undertook clinical trainings in Paediatrics in Paris, Assistance Publique Hopitaux de Paris. She became involved in Cystic Fibrosis during her fellowship under the supervision of Pr G Lenoir. She is now the head of the paediatric Cystic Fibrosis center, at the Necker-Enfants Malades Hospital. She has been engaged for several years in a programme of phenotype-genotype studies, using epithelial electrophysiological measurements. She works closely on a number of commercially-sponsored clinical trials in CF, largely involving novel small-molecules directed at CFTR function. She runs a clinic which accepts referrals from throughout France for questionable diagnosis for CF. She is involved in the European Clinical Trial Network as head of the standardization committee. She is the head of a research laboratory at INSERM whose main focus is CFTR interactome and and molecular mechanisms for specific CFTR mutations.

JoVE Journal Publications

ArticleTotal : 2
Year
Standardized Measurement of Nasal Membrane Transepithelial Potential Difference (NPD)
Publication title

Cited by 30

2018
2022

Other Publications

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

Journal of clinical microbiology| PubMed ID: 12354883

2002
Mycobacterium abscessus and children with cystic fibrosis.

Emerging infectious diseases| PubMed ID: 14720400

2003
Measurement of immunoglobulin G against Mycobacterial antigen A60 in patients with cystic fibrosis and lung infection due to Mycobacterium abscessus.

Clinical infectious diseases : an official publication of the Infectious Diseases Society of America| PubMed ID: 15614693

2005
2005
2006
2006
2006
Low bone mineral density in young children with cystic fibrosis.

American journal of respiratory and critical care medicine| PubMed ID: 17272788

2007
[Anti Pseudomonas aeruginosa antibiotic therapy in cystic fibrosis (exclusion of macrolides)].

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie| PubMed ID: 17370394

2006
2007
2007
[Recommendations for the management of bone demineralization in cystic fibrosis].

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie| PubMed ID: 18325750

2008
Bacterial contamination in the environment of hospitalised children with cystic fibrosis.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society| PubMed ID: 18550452

2008
2008
Pain in children and adults with cystic fibrosis: a comparative study.

Journal of pain and symptom management| PubMed ID: 19364632

2009
[Novel therapies for cystic fibrosis in 2009].

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie| PubMed ID: 19541091

2009
[Bone health in cystic fibrosis].

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie| PubMed ID: 19541106

2009
2009
Update on cystic fibrosis-related bone disease: a special focus on children.

Paediatric respiratory reviews| PubMed ID: 19651384

2009
2010
2010
2010
Guidelines on the early management of infants diagnosed with cystic fibrosis following newborn screening.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society| PubMed ID: 20605539

2010
2010
[French guidelines for sweat test practice and interpretation for cystic fibrosis neonatal screening].

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie| PubMed ID: 20719482

2010
2011
2012
Ivacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation.

European respiratory review : an official journal of the European Respiratory Society| PubMed ID: 23457167

2013
Biosynthesis of cystic fibrosis transmembrane conductance regulator.

The international journal of biochemistry & cell biology| PubMed ID: 24685677

2014
[National French guidelines for management of infants with cystic fibrosis].

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie| PubMed ID: 24815598

2014
2014
European Cystic Fibrosis Society Standards of Care: Best Practice guidelines.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society| PubMed ID: 24856775

2014
2014
Central venous thrombosis and thrombophilia in cystic fibrosis: A prospective study.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society| PubMed ID: 25107684

2015
2014
2014
Persistent Bordetella bronchiseptica infection in a child with cystic fibrosis: Relationship to bacterial phenotype.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society| PubMed ID: 25900817

2015
2015
2015
2016
2016
2016
Bioelectrical impedance in young patients with cystic fibrosis: Validation of a specific equation and clinical relevance.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society| PubMed ID: 27289197

2016
2016
Changes of CFTR functional measurements and clinical improvements in cystic fibrosis patients with non p.Gly551Asp gating mutations treated with ivacaftor.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society| PubMed ID: 27659740

2017
Analysis of nasal potential in murine cystic fibrosis models.

The international journal of biochemistry & cell biology| PubMed ID: 27717840

2016
Bone demineralization is improved by ivacaftor in patients with cystic fibrosis carrying the p.Gly551Asp mutation.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society| PubMed ID: 27745802

2016
French law: what about a reasoned reimbursement of serum vitamin D assays?

Geriatrie et psychologie neuropsychiatrie du vieillissement| PubMed ID: 27818369

2016
Diagnosis of Cystic Fibrosis in Screened Populations.

The Journal of pediatrics| PubMed ID: 28129810

2017
2017
[Management of infants whose diagnosis is inconclusive at neonatal screening for cystic fibrosis].

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie| PubMed ID: 28258861

2017
A multiple reader scoring system for Nasal Potential Difference parameters.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society| PubMed ID: 28465124

2017
2017
Multifocal fixed drug eruption to ceftazidime in a child with cystic fibrosis.

Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology| PubMed ID: 29047164

2018
2017
Guidelines for the clinical management and follow-up of infants with inconclusive cystic fibrosis diagnosis through newborn screening.

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie| PubMed ID: 29174009

2017
2018
2018
ECFS best practice guidelines: the 2018 revision.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society| PubMed ID: 29506920

2018
N1303K: Leaving no stone unturned in the search for transformational therapeutics.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society| PubMed ID: 30126793

2018
2018
2018
Might Brushed Nasal Cells Be a Surrogate for CFTR Modulator Clinical Response?

American journal of respiratory and critical care medicine| PubMed ID: 30326728

2019
2018
Predictive factors for lumacaftor/ivacaftor clinical response.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society| PubMed ID: 30595473

2019
Chronic Staphylococcus aureus Lung Infection Correlates With Proteogenomic and Metabolic Adaptations Leading to an Increased Intracellular Persistence.

Clinical infectious diseases : an official publication of the Infectious Diseases Society of America| PubMed ID: 30753350

2019
2019
2019
2019
2020
Real-Life Safety and Effectiveness of Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis.

American journal of respiratory and critical care medicine| PubMed ID: 31601120

2020
Cystic Fibrosis Diagnosis in Newborns, Children, and Adults.

Seminars in respiratory and critical care medicine| PubMed ID: 31679154

2019
Cystic fibrosis bone disease treatment: Current knowledge and future directions.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society| PubMed ID: 31679730

2019
Insights into the variability of nasal potential difference, a biomarker of CFTR activity.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society| PubMed ID: 31699569

2020
2019
CFTR: New insights into structure and function and implications for modulation by small molecules.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society| PubMed ID: 31759907

2020
Suppressing 'nonsense' in cystic fibrosis.

The Journal of physiology| PubMed ID: 31869855

2020
Impact of COVID-19 on people with cystic fibrosis.

The Lancet. Respiratory medicine| PubMed ID: 32304639

2020
Clinical response to lumacaftor-ivacaftor in patients with cystic fibrosis according to baseline lung function.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society| PubMed ID: 32591294

2021
Urinary Exosomes of Patients with Cystic Fibrosis Unravel CFTR-Related Renal Disease.

International journal of molecular sciences| PubMed ID: 32927759

2020
2021
2021
Exon identity influences splicing induced by exonic variants and in silico prediction efficacy.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society| PubMed ID: 33341408

2021
Arterial abnormalities identified in kidneys transplanted into children during the COVID-19 pandemic.

American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons| PubMed ID: 33346946

2021
2021
Modulators of CFTR. Updates on clinical development and future directions.

European journal of medicinal chemistry| PubMed ID: 33524685

2021
"Il faut continuer à poser des questions" patient reported outcome measures in cystic fibrosis: An anthropological perspective.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society| PubMed ID: 33648900

2021
2021
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.

Euro surveillance : bulletin Europeen sur les maladies transmissibles = European communicable disease bulletin| PubMed ID: 33797390

2021
2021
Involvement of CFTR in the pathogenesis of pulmonary arterial hypertension.

The European respiratory journal| PubMed ID: 33926975

2021
Antisense oligonucleotide-based drug development for Cystic Fibrosis patients carrying the 3849+10 kb C-to-T splicing mutation.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society| PubMed ID: 34226157

2021
2021
2021
Lumacaftor-ivacaftor effects on cystic fibrosis-related liver involvement in adolescents with homozygous F508 del-CFTR.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society| PubMed ID: 34454846

2022
2021
2021
2021
2022
Inflammation biomarkers in sputum for clinical trials in cystic fibrosis: current understanding and gaps in knowledge.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society| PubMed ID: 34772643

2021
2022
Reclassifying inconclusive diagnosis after newborn screening for cystic fibrosis. Moving forward.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society| PubMed ID: 34949556

2021
2022
2022