A5022662991- Cystic Fibrosis Research Advances
- Neonatal Respiratory Health Research
- Pediatric health and respiratory diseases
- Tracheal and airway disorders
- Respiratory viral infections research
- Nematode management and characterization studies
- Pneumonia and Respiratory Infections
- Asthma and respiratory diseases
- Antibiotic Resistance in Bacteria
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Atomic and Subatomic Physics Research
- Antifungal resistance and susceptibility
- Inhalation and Respiratory Drug Delivery
- Antibiotics Pharmacokinetics and Efficacy
- Regulation of Appetite and Obesity
- Respiratory Support and Mechanisms
- Plant Pathogenic Bacteria Studies
- Chronic Obstructive Pulmonary Disease (COPD) Research
- Oral and gingival health research
- Neuroscience of respiration and sleep
- Obstructive Sleep Apnea Research
- Biochemical Analysis and Sensing Techniques
- Cardiovascular and Diving-Related Complications
- Digestive system and related health
- Infections and bacterial resistance
Centre Hospitalier Universitaire de Bordeaux
2016-2025
Hôpital Pellegrin
2013-2025
Inserm
2013-2024
Université de Bordeaux
2017-2024
Centre de Recherche Cardio-Thoracique de Bordeaux
2020-2024
Bordeaux Imaging Center
2024
Clinical Investigation Center Plurithematic Tours
2017-2024
Hôpital des Enfants
2006-2024
Maison des Sciences de l’Homme de Dijon
2024
Hôpital Jeanne de Flandre
2024
Rationale: Elexacaftor-tezacaftor-ivacaftor is a CFTR (cystic fibrosis [CF] transmembrane conductance regulator) modulator combination, developed for patients with CF at least one Phe508del mutation. Objectives: To evaluate the effects of elexacaftor-tezacaftor- ivacaftor in and advanced respiratory disease. Methods: A prospective observational study, including all aged ⩾12 years percent-predicted FEV1 (ppFEV1) <40 who initiated elexacaftor-tezacaftor-ivacaftor from December 2019 to August...
Rationale: Lumacaftor-ivacaftor is a CFTR (cystic fibrosis transmembrane conductance regulator) modulator combination recently approved for patients with cystic (CF) homozygous the Phe508del mutation.Objectives: To evaluate safety and effectiveness of lumacaftor-ivacaftor in adolescents (≥12 yr) adults (≥18 real-life postapproval setting.Methods: The study was conducted 47 CF reference centers France. All who initiated from January 1 to December 31, 2016, were eligible. Patients evaluated...
Highly effective CFTR modulators, such as elexacaftor/tezacaftor/ivacaftor (ETI), herald a new era in therapeutic strategy of cystic fibrosis (CF). ETI impact on glucose tolerance remains controversial. All the participants underwent baseline oral test (OGTT) before initiation (M0) and 12 months (M12), at 24 if possible. The cohort was stratified two subgroups based OGTT: normal (NGT) abnormal (AGT) defined by impaired fasting or diabetes not requiring insulin treatment. We included 106...
Leptin deficiency results in a complex obesity phenotype comprising both hyperphagia and lowered metabolism. The results, at least part, from the absence of induction by leptin melanocyte stimulating hormone (MSH) secretion hypothalamus; MSH normally then binds to melanocortin-4 receptor expressing neurons inhibits food intake. basis for reduced metabolic rate has been unknown. Here we show that administered leptin-deficient (ob/ob) mice large increase peripheral levels; further,...
Cystic fibrosis (CF) is caused by compound heterozygosity or homozygosity of CF transmembrane conductance regulator gene (CFTR) mutations. Phenotypic variability associated with certain mutations makes genetic counselling difficult, notably for R117H, whose disease phenotype varies from asymptomatic to classical CF. The high frequency R117H observed in newborn screening has also introduced diagnostic dilemmas. aim this study was evaluate the penetrance order improve clinical practice.The...
Cystic fibrosis (CF) is a systemic genetic disease that leads to pulmonary and digestive disorders. In the majority of CF patients, intestine site chronic inflammation microbiota disturbances. The link between gut dysbiosis still poorly understood. main objective this study was assess composition in children depending on their intestinal inflammation. We collected fecal samples from 20 with CF. Fecal calprotectin levels were measured analyzed by 16S rRNA sequencing. observed associated...
Forced spirometry is the gold standard to assess lung function, but its accessibility may be limited. By contrast, home telemonitoring allows a multi-weekly function follow-up real-life adherence, reliability, and variability according age have been poorly studied in patients with CF (PwCF). We aimed compare reliability of between children, teenagers adults CF. This observational study included PwCF followed for six months whom (i.e, forced expiratory volume maximum 1 s (FEV1), vital...
The diagnosis of cystic fibrosis (CF) is based on a characteristic clinical picture in association with sweat chloride (Cl(-)) concentration greater than 60 mmol/L or the identification two CF-causing mutations. A challenging problem significant number children for whom no definitive possible because they present symptoms suggestive CF, level intermediate range between 30 and mmol/L, only one identified mutation.To investigate function transmembrane conductance regulator (CFTR) protein...
Purpose To assess the diagnostic accuracy of mucus contrast characterization by using magnetic resonance (MR) imaging to discriminate allergic bronchopulmonary aspergillosis (ABPA) in cystic fibrosis (CF). Materials and Methods The study was approved local Ethics Committee, all patients or their parents gave written informed consent. One hundred ten consecutive with CF were screened between January 2014 July 2015. All underwent a non-contrast material-enhanced MR protocol that included...
Adult mouse mutants homozygous for an engineered proopiomelanocortin (POMC)-null allele lack macroscopically distinct adrenal glands and circulating hormones. To understand the basis this defect, we compared development of primordia in POMC-null mice littermate controls. mutant are born with that morphologically indistinguishable from those their wild-type littermates. However, cells fail to proliferate postnatally adrenals atrophy until they have disappeared adult. While present,...
Background Chest computed tomography (CT) remains the imaging standard for demonstrating cystic fibrosis (CF) airway structural disease in vivo . However, visual scoring systems as an outcome measure are time consuming, require training and lack high reproducibility. Our objective was to validate a fully automated artificial intelligence (AI)-driven system of CF lung severity. Methods Data were retrospectively collected three reference centres, between 2008 2020, 184 patients aged 4–54...
Objectives Lumacaftor-ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) modulator known to improve clinical status in people with (CF). This study aimed assess lung structural changes after one year of lumacaftor-ivacaftor treatment, and use unsupervised machine learning identify morphological phenotypes disease that are associated response lumacaftor-ivacaftor. Methods Adolescents adults CF from the French multicenter real-world prospective observational evaluating...
Background Lung MRI with ultrashort echo times (UTEs) enables high-resolution and radiation-free morphologic imaging; however, its image quality is still lower than that of CT. Purpose To assess the clinical applicability synthetic CT images generated from UTE by a generative adversarial network (GAN). Materials Methods This retrospective study included patients cystic fibrosis (CF) who underwent both on same day at one six institutions between January 2018 December 2022. The two-dimensional...
<h3>Background</h3> A challenging problem arising from cystic fibrosis (CF) newborn screening is the significant number of infants with hypertrypsinaemia (HIRT) sweat chloride levels in intermediate range and only one or no identified CF-causing mutations. <h3>Objectives</h3> To investigate diagnostic value for CF assessing transmembrane conductance regulator (CFTR) protein function by measuring nasal potential difference children HIRT. <h3>Methods</h3> specially designed protocol was used...
Cystic fibrosis-related liver disease (CFLD) can develop silently in early life and approximately 10% of children with cystic fibrosis (CF) become cirrhotic before adulthood. Clinical, biological, ultrasound criteria used to define CFLD often reveal involvement at an advanced stage. The aim this retrospective study was assess the progression stiffness measurement (LSM) pediatric patients CF.The change LSM, expressed as kPa/year %/year, measured using transient elastography (Fibroscan) 82 CF...
A chronic intestinal inflammation may occur in patients with cystic fibrosis (CF), while no therapeutic management is proposed. Although Lumacaftor/Ivacaftor well-known to modulate the defective transmembrane conductance regulator (CFTR) protein lungs, data are available on impact of this treatment CF disorders. We, therefore, investigated evolution after initiation adolescents (median follow-up: 336 days [IQR: 278;435]). Median fecal calprotectin concentrations decreased significantly (102...