Bronchial or Laryngeal Obstruction Induced by Exercise?

A child suspected of exercise-induced laryngeal obstruction and asthma is examined by laryngoscopy and respiratory resistance (Rrs) after exercise challenge. Immediately at exercise cessation, the visualized adduction of the larynx in inspiration is reflected in a paroxystic increase in Rrs. While normal breathing has apparently resumed later on during recovery from exercise, the pattern of Rrs in inspiration is observed to reoccur following a deep breath or swallowing. The procedure may thus help diagnosing the site of exercise-induced obstruction when laryngoscopy is not available and identify re-inducers of laryngeal dysfunction

Paediatric long term continuous positive airway pressure and noninvasive ventilation in France: A cross-sectional study

International audienceObjective: To describe the characteristics of children treated with long term continuous positive airway pressure (CPAP) or noninvasive ventilation (NIV) in France.Design: Cross-sectional national survey.Setting: Paediatric CPAP/NIV teams of 28 tertiary university hospitals in France.Patients: Children aged <20 years treated with CPAP/NIV since at least 3 months on June 1st, 2019.Intervention: An anonymous questionnaire was filled in for every patient.Results: The data of 1447 patients (60% boys), mean age 9.8 ± 5.8 years were analysed. The most frequent underlying disorders were: upper airway obstruction (46%), neuromuscular disease (28%), disorder of the central nervous system (13%), cardiorespiratory disorder (7%), and congenital bone disease (4%). Forty-five percent of the patients were treated with CPAP and 55% with NIV. Treatment was initiated electively for 92% of children, while 8% started during an acute illness. A poly(somno)graphy (P(S)G) was performed prior to treatment initiation in 26%, 36% had a P(S)G with transcutaneous carbon dioxide monitoring (PtcCO2), while 23% had only a pulse oximetry (SpO2) with PtcCO2 recording. The decision of CPAP/NIV initiation during an elective setting was based on the apnea-hypopnea index (AHI) in 41% of patients, SpO2 and PtcCO2 in 25% of patients, and AHI with PtcCO2 in 25% of patients. Objective adherence was excellent with a mean use of 7.6 ± 3.2 h/night. Duration of CPAP/NIV was 2.7 ± 2.9 years at the time of the survey.Conclusion: This survey shows the large number of children treated with long term CPAP/NIV in France with numerous children having disorders other than neuromuscular disease

Long term noninvasive ventilation and continuous positive airway pressure in children with neuromuscular diseases in France

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Clinical problems in rare interstitial lung diseases

International audienceIntroduction: Interstitial lung disease ( LD) in children (chlLD) is rare and Often severe. This study aims at analyzing the ep demiology of chlLD in France from 2000 to 2022.Methods: This study was retrospective and multicentric, A questionnaire was sent to all the RespiRare centers to collect the clinical, radiological, biological, histological and genetic data of the patients.Results: 617 patients (0-18 years) were included in 42 centers. 84 patients were excluded. The median age at diagnosis was 0.3 years with 17% of familial forms, The main investigations performed were: chest CT scan (92%), bronchoalveolar avage (52%), genetic ana ysis (78%), lung biopsy (23%). The main treatments were: corticosteroids (93%), oxygen therapy (52.2%), enteral nutrition (29%), hydroxychloroqu•ne (16%), azThromycin (26%), immunosuppressive drugs (210/0). The follovFup time was from O to 18,9 years (median duration 3,5years). The survival rate at 5 years was 68%. The overall incidence and preva ence were estimated at 38/million and 35/million children respectively.Conclusion: This arge chlLD epidemiological study confirms the Span •sh data with a higher incidence and prevalence than previous y described. The arge amount Of phenotypic data collected will allow better understanding ch LD and harmonizing their management

Clinical problems in rare interstitial lung diseases

International audienceIntroduction: Interstitial lung disease ( LD) in children (chlLD) is rare and Often severe. This study aims at analyzing the ep demiology of chlLD in France from 2000 to 2022.Methods: This study was retrospective and multicentric, A questionnaire was sent to all the RespiRare centers to collect the clinical, radiological, biological, histological and genetic data of the patients.Results: 617 patients (0-18 years) were included in 42 centers. 84 patients were excluded. The median age at diagnosis was 0.3 years with 17% of familial forms, The main investigations performed were: chest CT scan (92%), bronchoalveolar avage (52%), genetic ana ysis (78%), lung biopsy (23%). The main treatments were: corticosteroids (93%), oxygen therapy (52.2%), enteral nutrition (29%), hydroxychloroqu•ne (16%), azThromycin (26%), immunosuppressive drugs (210/0). The follovFup time was from O to 18,9 years (median duration 3,5years). The survival rate at 5 years was 68%. The overall incidence and preva ence were estimated at 38/million and 35/million children respectively.Conclusion: This arge chlLD epidemiological study confirms the Span •sh data with a higher incidence and prevalence than previous y described. The arge amount Of phenotypic data collected will allow better understanding ch LD and harmonizing their management

Clinical problems in rare interstitial lung diseases

International audienceIntroduction: Interstitial lung disease ( LD) in children (chlLD) is rare and Often severe. This study aims at analyzing the ep demiology of chlLD in France from 2000 to 2022.Methods: This study was retrospective and multicentric, A questionnaire was sent to all the RespiRare centers to collect the clinical, radiological, biological, histological and genetic data of the patients.Results: 617 patients (0-18 years) were included in 42 centers. 84 patients were excluded. The median age at diagnosis was 0.3 years with 17% of familial forms, The main investigations performed were: chest CT scan (92%), bronchoalveolar avage (52%), genetic ana ysis (78%), lung biopsy (23%). The main treatments were: corticosteroids (93%), oxygen therapy (52.2%), enteral nutrition (29%), hydroxychloroqu•ne (16%), azThromycin (26%), immunosuppressive drugs (210/0). The follovFup time was from O to 18,9 years (median duration 3,5years). The survival rate at 5 years was 68%. The overall incidence and preva ence were estimated at 38/million and 35/million children respectively.Conclusion: This arge chlLD epidemiological study confirms the Span •sh data with a higher incidence and prevalence than previous y described. The arge amount Of phenotypic data collected will allow better understanding ch LD and harmonizing their management