33 results match your criteria: "Centre de Référence et de Compétence en Mucoviscidose[Affiliation]"
Eur Arch Otorhinolaryngol
December 2024
Service d'Explorations Fonctionnelles Pédiatriques, Hôpital d'Enfants, Centre Hospitalier Universitaire de Nancy, Nancy, France.
Background: Oximetry was proposed as an abbreviated exam, easily accepted by the child, for the diagnosis of obstructive sleep apnea (OSA) for children located in regions where access to pediatric sleep labs is limited. The objective of this study was to determine the diagnostic value of the oxygen desaturation index (ODI), the number of ≥ 3% oxygen desaturations per hour of recording, obtained by portable oximetry performed in parallel with video-polysomnography (PSG), in a cohort of children, with and without comorbidities, referred for OSA.
Methods: Data from portable oximetry performed in parallel with PSG were prospectively collected.
Sleep Med
January 2025
DevAH, Faculté de Médecine, Université de Lorraine, France; Service d'Explorations Fonctionnelles Pédiatriques, Hôpital d'Enfants, Centre Hospitalier Universitaire de Nancy, France. Electronic address:
Polysomnography (PSG) is the gold standard for diagnosing obstructive sleep apnea (OSA) in children but not always available. Abbreviated exams exist, such as respiratory polygraphy (RP), but are less accurate for OSA diagnosis. Video recording (video-RP) may provide a more precise estimation of the total sleep time (TST) compared to RP alone.
View Article and Find Full Text PDFJ Clin Sleep Med
July 2024
Service de Pneumologie Infantile, Allergologie et Centre De Référence En Mucoviscidose, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Bron, France.
Study Objectives: Sleep laboratory polysomnography is the gold standard for obstructive sleep apnea (OSA) diagnosis in infants, but its access remains limited. Oximetry-capnography is another simple and widely used tool that can provide information on the presence of desaturations and alveolar hypoventilation. However, its reliability is debated.
View Article and Find Full Text PDFSleep Med
February 2023
EA3450-DevAH, Faculté de Médecine, Université de Lorraine, France; Service de Médecine Infantile, Centre Hospitalier Universitaire de Nancy, France.
Background: Ambulatory exams were preferred in children during the COVID-19 pandemic. Polysomnography (PSG), the gold standard for obstructive sleep apnea (OSA) diagnosis, requires several leads and sensors to be attached to the child's body. Children are more comfortable with respiratory polygraphic (RP) recording, which needs fewer sensors.
View Article and Find Full Text PDFSleep Med
December 2022
Service d'épileptologie clinique, des troubles du sommeil et de neurologie fonctionnelle de l'enfant, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Lyon, France; U1028, Lyon Neuroscience Research Center (CNRL), University Lyon 1, Lyon, France. Electronic address:
Obstructive sleep apnea syndrome (OSAS) treatment has been shown to improve cardiac behavioral and cognitive functions in typically developing children. Early OSAS diagnosis in children with Down syndrome (DS) would be important to prevent its complications, especially cognitive ones, but remains overlooked. The main objective of our study was to assess the cognitive function of children with DS, with and without OSAS.
View Article and Find Full Text PDFRev Mal Respir
February 2022
CRCM pédiatrie, UTEP, CHU Grenoble-Alpes, CS 10217, 38043 Grenoble cedex, France; Groupe d'éducation thérapeutique en mucoviscidose (GETHEM), filière Muco-CFTR, France.
Introduction: In 2018, 55.4% of the 7180 French cystic fibrosis (CF) patients were adults. Our study was aimed at identifying young adult patients' needs and those of their parents when the young adults arrived in an adult CF center.
View Article and Find Full Text PDFJ Clin Sleep Med
January 2022
Service d'Épileptologie Clinique, des Troubles du Sommeil et de Neurologie Fonctionnelle de l'Enfant, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, and U1028, CNRL, Université de Lyon 1, Lyon, France.
Study Objectives: Children with Down syndrome (DS) are at risk of obstructive sleep apnea (OSA), but the access to sleep lab polysomnography (PSG) is limited. Simplified techniques are needed, such as polygraphy coupled with pulse transit time (PTT-PG) that detects respiratory events and the total autonomic arousals index (PTTAI). Our objective was to assess the ability of PTT-PG compared with PSG to diagnose OSA in children with DS.
View Article and Find Full Text PDFSleep Med
August 2020
Service d'épileptologie clinique, des troubles du sommeil et de neurologie fonctionnelle de l'enfant, Hôpital Femme Mère Enfants, Hospices Civils de Lyon, Bron, France; INSERM, U1028, CNRS, UMR5292, Centre de Recherche en Neurosciences de Lyon, Lyon, France.
Objective/background: Currently, obstructive sleep apnea syndrome (OSAS) management in Robin sequence (RS) infants has not been standardized. Sleep laboratory polysomnography (PSG) is the gold standard for OSAS diagnosis, however, access is restricted. This study aimed to compare the respiratory indexes measured in a sleep laboratory using PSG as well as a possible alternative, polygraphy (PG).
View Article and Find Full Text PDFFront Immunol
November 2020
Aix-Marseille Univ, IRD, APHM, MEPHI, IHU Méditerranée Infection, MEPHI, Marseille, France.
Allergic bronchopulmonary mycosis (ABPM) is an underestimated allergic disease due to fungi. Most reported cases are caused by (Af) and are referred to as allergic bronchopulmonary aspergillosis (ABPA). The main risk factor of ABPA is a history of lung disease, such as cystic fibrosis, asthma, or chronic obstructive pulmonary disease.
View Article and Find Full Text PDFJ Clin Sleep Med
July 2020
Service Epilepsie, Sommeil, Explorations Fonctionnelles Neurologiques Pédiatriques, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Bron, France.
Study Objectives: Due to a limited number of pediatric sleep centers, the aim was to test the feasibility of ambulatory polysomnography (PSG-home) in a group of French children suspected of OSA.
Methods: Children undergoing one-night PSG-home, with the device installed at the pediatric sleep physician's office, were prospectively included. General failure was considered when PSG-home recording captured < 5 h of artifact-free sleep or when ≥ 1 channel (nasal flow, thoraco-abdominal belts, oximetry) presented artifacts > 75% of the recording time.
Rev Mal Respir
May 2019
(GETHEM), CRCM pédiatrie-UTEP, groupe d'éducation thérapeutique en mucoviscidose, CHU de Grenoble Alpes, avenue Maquis-du-Grésivaudan, 38700 La Tronche, France.
Introduction: SAFETIM-APP compiled an inventory of professional practice in the 45 French cystic fibrosis reference centres (CFRC), between February 2015 and December 2016, related to the transition of adolescents with cystic fibrosis to adult centres.
Method: This multicentre cross-sectional study addressed the modalities of the transition in CFRCs and proposed a list of items that could be used to establish quality criteria. Quantitative analysis of the criteria and a qualitative analysis of the transition procedure were carried out.
Allergy
September 2019
Aix-Marseille Univ, IRD, APHM, MEPHI, IHU Méditerranée Infection, MEPHI, Marseille, France.
Sleep Med
February 2019
Service d'épileptologie clinique, des troubles du sommeil et de neurologie fonctionnelle de l'enfant, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Bron, France; U1028, CNRL, Université de Lyon 1, France.
Objective/background: Obstructive sleep apnea syndrome (OSA) is frequent in Pierre Robin sequence (PRS) infants. Prone positioning (PP) is commonly recommended but has never been studied by polysomnography (PSG). This study aimed to evaluate the impact of the PP on sleep and breathing outcomes measured by PSG.
View Article and Find Full Text PDFBMC Med
August 2018
Centre de Ressources et de Compétence en Mucoviscidose, Hôpital Necker-Enfants Malades, AP-HP, Paris, France.
Clin Exp Allergy
July 2018
Service de Pneumologie et Allergologie Pédiatriques, Hôpital Universitaire Necker-Enfants Malades, Paris, France.
Background: The influence of airway remodelling and inflammation in preschoolers with severe recurrent wheeze on asthma outcomes is poorly understood.
Objective: To assess their association with asthma symptoms and lung function at school age.
Methods: Preschoolers (38.
J Cyst Fibros
September 2018
Aix-Marseille Univ, IRD, IHU Méditerranée Infection, MEPHI, Marseille, France. Electronic address:
Pulmonary and systemic antifungal immunity influences quality of life and survival of people with cystic fibrosis. Aspergillus fumigatus (Af) induces specific IgG and IgE. Mast cells respond to IgE, IgG and direct interactions with Af.
View Article and Find Full Text PDFFront Immunol
August 2017
Aix Marseille Univ, IRD, INSERM, SESSTIM UMR 912, Faculté de Médecine campus Timone, Marseille, France.
Molecular-based allergy diagnosis yields multiple biomarker datasets. The classical diagnostic score for allergic bronchopulmonary aspergillosis (ABPA), a severe disease usually occurring in asthmatic patients and people with cystic fibrosis, comprises succinct immunological criteria formulated in 1977: total IgE, anti- () IgE, anti- "precipitins," and anti- IgG. Progress achieved over the last four decades led to multiple IgE and IgG(4) biomarkers available with quantitative, standardized, molecular-level reports.
View Article and Find Full Text PDFCurr Opin Pulm Med
November 2017
Centre de Ressources et de Compétence en Mucoviscidose, Hôpital Necker, Université Paris Sorbonne, Paris, France.
Purpose Of Review: Life expectancy is increasing in cystic fibrosis and new aspects of the disease have to be taken into account in cystic fibrosis care.
Recent Findings: Pain is encountered among 70% of adult and pediatric patients with cystic fibrosis. This symptom is underestimated by the multidisciplinary team.
Allergy
November 2017
Aix-Marseille Univ, INSERM UMR 1067, CNRS UMR 7333, Marseille, France.
A wide spectrum of pathological conditions may result from the interaction of Aspergillus fumigatus and the immune system of its human host. Allergic bronchopulmonary aspergillosis is one of the most severe A. fumigatus-related diseases due to possible evolution toward pleuropulmonary fibrosis and respiratory failure.
View Article and Find Full Text PDFArch Pediatr
April 2017
Laboratoire de génétique moléculaire, hôpital Cochin, 75014 Paris, France.
Neonatal screening for cystic fibrosis (CF) may detect infants with elevated immunoreactive trypsinogen (IRT) levels but with inconclusive sweat tests and/or DNA results. This includes cases associating (1) either the presence of at most one CF-causing mutation and sweat chloride values between 30 and 59mmol/L or (2) two CFTR mutations with at least one of unknown pathogenicity and a sweat chloride below 60mmol/L. This encompasses different clinical situations whose progression cannot be predicted.
View Article and Find Full Text PDFJ Clin Microbiol
November 2016
Laboratoire de Parasitologie-Mycologie, Centre Hospitalier Universitaire, Rouen, France
The aim of this work was to document molecular epidemiology of Rasamsonia argillacea species complex isolates from cystic fibrosis (CF) patients. In this work, 116 isolates belonging to this species complex and collected from 26 CF patients and one patient with chronic granulomatous disease were characterized using PCR amplification assays of repetitive DNA sequences and electrophoretic separation of amplicons (rep-PCR). Data revealed a clustering consistent with molecular species identification.
View Article and Find Full Text PDFAllergy
November 2016
Laboratoire d'Immunologie, Hôpital de La Conception, APHM Assistance Publique Hôpitaux de Marseille, Marseille, France.
Aspergillus fumigatus is the causative agent of allergic broncho-pulmonary aspergillosis. Prompt and accurate diagnosis may be difficult to achieve with current clinical and laboratory scores, which do not include immune responses to recombinant A. fumigatus allergens.
View Article and Find Full Text PDFJ Cyst Fibros
September 2015
Assistance-Publique Hôpitaux de Paris, Hôpital Necker, Service de Pneumologie-Allergologie Pédiatrique et Centre de Ressources et de Compétences en Mucoviscidose, Paris, France; Université Paris Descartes, Paris Sorbonne Cite, Paris, France.
Backgrounds: Recent reports have pointed the low vaccine coverage in patients with chronic diseases. Data are lacking in patients with cystic fibrosis (CF). Gaining more information on coverage both for mandatory vaccines and those more specifically recommended would help to optimize care of these patients.
View Article and Find Full Text PDFJ Cyst Fibros
January 2015
EA3647, UFR des Sciences de la Santé Simone Veil, Université de Versailles Saint Quentin en Yvelines (UVSQ), Guyancourt, France; Laboratoire de Microbiologie, Hôpital Raymond Poincaré, Assistance Publique Hôpitaux de Paris (AP-HP), Garches, France; Laboratoire de Microbiologie, Hôpital Ambroise Paré, AP-HP, Boulogne-Billancourt, France.
Background: Mycobacterium massiliense is closely related to Mycobacterium abscessus and is also a frequent cause of mycobacterial lung disease in patients with cystic fibrosis (CF). There has been no previous investigation of possible differences between M. massiliense and M.
View Article and Find Full Text PDFVaccine
July 2014
Université Paris Descartes, Paris Sorbonne Cité, Paris, France; Assistance-Publique Hôpitaux de Paris, Hôpital Necker, Service de Pneumologie- Allergologie Pediatrique et Centre de Ressources et de Compétences en Mucoviscidose, Paris, France; INSERM U 1151, Paris, France. Electronic address:
Influenza vaccination is recommended in cystic fibrosis patients. The objective of this study was to assess the immunogenicity of vaccination against 2009 pandemic A/H1N1 influenza and to study the factors associated with the immune response in patients with cystic fibrosis. 122 patients with cystic fibrosis were enrolled in a prospective study and received 1 dose of 2009/H1N1v adjuvanted vaccine, or for children <2 years and lung-transplanted patients, two doses of non-adjuvanted 2009/H1N1v vaccine administered 21 days apart.
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