33 results match your criteria: "Centre de Référence et de Compétence en Mucoviscidose[Affiliation]"

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.

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Evaluating the added-value of video recording in respiratory polygraphy for the diagnosis of obstructive sleep apnea in children.

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.

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Gas exchange parameters for the prediction of obstructive sleep apnea in infants.

J 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.

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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.

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Neurocognitive evaluation of children with down syndrome and obstructive sleep apnea syndrome.

Sleep 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.

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[How can the transition of adolescents from a children's to an adult CF center be improved? Analysis of adolescents' and parents' needs during the post-transfer period].

Rev 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.

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Pulse transit time as a diagnostic test for OSA in children with Down syndrome.

J 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.

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The role of sleep laboratory polygraphy in the evaluation of obstructive sleep apnea syndrome in Robin infants.

Sleep 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).

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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.

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Feasibility of parent-attended ambulatory polysomnography in children with suspected obstructive sleep apnea.

J 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.

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[Quality criteria for the transition to adult care in French CF centers - results from the SAFETIM APP study?].

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.

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Impact of prone positioning in infants with Pierre Robin sequence: a polysomnography study.

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.

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Article Synopsis
  • The original article has inaccuracies in Table 1, specifically related to oligonucleotide sequences.
  • These errors also impact the readthrough values presented in the same table.
  • Corrections need to be made to ensure the data is accurate and reliable.
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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.

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Mast cell tryptase changes with Aspergillus fumigatus - Host crosstalk in cystic fibrosis patients.

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.

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Multivariate Analysis As a Support for Diagnostic Flowcharts in Allergic Bronchopulmonary Aspergillosis: A Proof-of-Concept Study.

Front 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.

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Pain is an underestimated symptom in cystic fibrosis.

Curr 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.

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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.

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

Arch 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.

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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.

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Aspergillus fumigatus components distinguish IgE but not IgG4 profiles between fungal sensitization and allergic broncho-pulmonary aspergillosis.

Allergy

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.

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Vaccine coverage in CF children: A French multicenter study.

J 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.

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Comparing Mycobacterium massiliense and Mycobacterium abscessus lung infections in cystic fibrosis patients.

J 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.

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

Vaccine

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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