Epidemiology of childhood interstitial lung disease in France: the RespiRare cohort.

INTRODUCTION: Interstitial lung disease in children (chILD) are rare and mostly severe lung diseases. Very few epidemiological data are available in limited series of patients. The aim of this study was to assess the prevalence and incidence of chILD in France. METHODS: We performed within the RespiRare network a multicentre retrospective observational study in patients with chILD from 2000 to 2022 and a prospective evaluation of chILD's incidence between February 2022 and 2023. RESULTS: chILD was reported in 790 patients in 42 centres. The estimated 2022 prevalence in France was 44 /million children (95% CI 40.76 to 47.46) and the computed incidence was 4.4 /million children (95% CI 3.44 to 5.56). The median age at diagnosis was 3 months with 16.9% of familial forms. Lung biopsy and genetic analyses were performed in 23.4% and 76.9%, respectively. The most frequent chILD aetiologies in the <2 years group were surfactant metabolism disorders (16.3%) and neuroendocrine cell hyperplasia of infancy (11.8%), and in the 2-18 years group diffuse alveolar haemorrhage (12.2%), connective tissue diseases (11.4%), hypersensitivity pneumonitis (8.8%) and sarcoidosis (8.8%). The management included mainly oxygen therapy (52%), corticosteroid pulses (56%), oral corticosteroids (44%), azithromycin (27.2%), enteral nutrition (26.9%), immunosuppressants (20.3%) and hydroxychloroquine (15.9%). The 5-year survival rate was 57.3% for the patients diagnosed before 2 years and 86% between 2 and 18 years. CONCLUSION: This large and systematic epidemiological study confirms a higher incidence and prevalence of chILD than previously described. In order to develop international studies, efforts are still needed to optimise the case collection and to harmonise diagnostic and management practices.

Assessment of a Multiplex LAMP Assay (Eazyplex® CSF Direct M) for Rapid Molecular Diagnosis of Bacterial Meningitis: Accuracy and Pitfalls.

Background: Automated molecular panels are attractive tools for improving early meningitis diagnosis. This study assessed the Eazyplex® CSF direct M panel (EP), a multiplex real-time Loop-Mediated Isothermal Amplification assay. Methods: From December 2016 to December 2019, cerebrospinal fluid (CSF) samples were routinely tested with the EP V1.0. CSF parameters and microbiological and clinical data were retrospectively collected. Results: Out of 230 CSF samples, the EP yielded positive, negative, and invalid results for 32 (13.9%) (16 N. meningitidis, nine S. pneumoniae, two S. agalactiae, two E. coli, two H. influenzae, one L. monocytogenes), 182 (79.1%), and 16 (7%) samples, respectively. Among the positive samples, 14 (44%) remained negative in culture (antibiotic therapy before lumbar puncture (n = 11), meningococcal meningitis (n = 3)). High CSF protein concentrations and cellularity were associated with LAMP inhibition, counteracted by centrifugation. The automated software yielded 13 false positive and five false negative results. Amplification curve analysis was necessary and enabled the attainment of positive (PPA) and negative percentage agreement and positive and negative predictive values of 91.4%, 100%, 100%, and 98.3%. Three false negative results remained (two E. coli and one N. meningitidis). E. coli presented the poorest PPA (50%). Conclusion: This work confirms the strong performance of the EP, of particular interest in cases of antibiotic therapy before lumbar puncture.

Retrospective audit of guidelines for investigation and treatment of bronchiolitis: a French perspective.

INTRODUCTION: As the most recent French bronchiolitis guidelines were published in 2000, there is a current overinvestigation and an overtreatment of infants hospitalised with bronchiolitis in France. In 2012, the Group of Pediatric University Hospitals in Western France ('HUGO') proposed new evidence-based clinical practice guidelines in keeping with the latest international guidelines. We hypothesise that the implementation of these guidelines contributed to the quality improvement of the management of bronchiolitis in our hospital. The aim of this study was to determine the impact of these guidelines on the management of bronchiolitis inpatients. METHODS: This retrospective before/after study design was conducted in the general paediatric unit of a tertiary care French hospital, looking at 1 year before (ie, the winter of 2011-2012) and 1 year after (ie, the winter of 2013-2014) the implementation of the guidelines. Two hundred and eighty bronchiolitis inpatients, all less than 1 year of age, 115 in 2011-2012 and 165 in 2013-2014, were included. The primary outcome we sought to evaluate was the proportion of children administered a diagnostic test associated with a treatment not routinely recommended by the guidelines. As balancing measures, we evaluated the length of stay, the intensive care unit transfer and the readmission rates. RESULTS: Following implementation of the guidelines, use of any given treatment associated with a diagnostic test was reduced by 66% (p<0.001). There were major decreases in the use of chest X-ray (86%vs26%, p<0.001), antibiotics (38%vs13%, p<0.001) and corticosteroids (10%vs3%, p=0.011). Balancing measures were not significantly different. CONCLUSIONS: HUGO guidelines were effective at reducing the administration of unnecessary diagnostic tests and medications. This study was the first step in convincing French paediatricians to streamline their practices until updated national guidelines are published.