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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.
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Background: Breastfeeding is a protective factor against respiratory and intestinal infections in developing countries. In developed countries, proof of this protection is more difficult to show. The objective of the study is to compare the proportion of children breastfed during their first year in groups of children with infectious pathologies supposedly prevented by breastfeeding and children free of these infectious pathologies. Method: Questionnaires about diet, socio-demographic data and the motive for consultation were given to the parents upon arrival in the paediatric emergency departments of 5 hospitals located in Pays de Loire (France) in 2018 and 2019. Children with lower respiratory tract infections, acute gastroenteritis and acute otitis media were included in the case group (A), children admitted for other reasons were included in the same control group (B). Breastfeeding was classified as exclusive or partial. Results: During the study period, 741 infants were included, of which 266 (35.9%) in group A. In this group, children were significantly less likely to have been breastfed at the time of admission than children in group B: for example, for children under 6 months, 23.3% were currently breastfed in group A, vs. 36.6% (weaned BF or formula diet) in group B [OR = 0.53 (0.34-0.82); p = 0.004]. Similar results were found at 9 and 12 months. After taking into account the age of the patients, the same results were confirmed with an aOR = 0.60 (0.38-0.94) (p = 0.02) at 6 months, but with when considering six variables six variables, aOR was not significative aOR = 0.65 (0.40-1.05); p = 0.08), meaning that factors such as the childcare out of home, socio-professional categories, and the pacifier decrease the protective effect of breastfeeding. Sensitivity analyses (age-matching, analysis by type of infection) showed the same protection effect provided by breastfeeding when it was pursued for at least 6 months and also that the protective effect of breastfeeding is especially true against gastro-enteritis. Conclusion: Breastfeeding is a protective factor against respiratory, gastrointestinal and ear infections when pursued at least 6 months after birth. Other factors such as collective childcare, pacifiers and low parental professional status can reduce the protective effect of breastfeeding.
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BACKGROUND: Pulmonary hemosiderosis is a rare and complex disease in children. A previous study from the French RespiRare® network led to two important findings: 20% of the children presented with both pulmonary hemosiderosis and Down syndrome (DS), and at least one tested autoantibody was found positive in 50%. This study investigates the relationships between pulmonary hemosiderosis and DS. METHODS: Patients younger than 20 years old and followed for pulmonary hemosiderosis were retrieved from the RespiRare® database. Clinical, biological, functional, and radiological findings were collected, and DS and non-DS patients' data were compared. RESULTS: A total of 34 patients (22 girls and 12 boys) were included, among whom nine (26%) presented with DS. The mean age at diagnosis was 4.1 ± 3.27 years old for non-DS and 2.9 ± 3.45 years old for DS patients. DS patients tended to present a more severe form of the disease with an earlier onset, more dyspnoea at diagnosis, more frequent secondary pulmonary hypertension, and an increased risk of fatal evolution. CONCLUSIONS: DS patients have a higher risk of developing pulmonary hemosiderosis, and the disease seems to be more severe in this population. This could be due to the combination of an abnormal lung capillary bed with fragile vessels, a higher susceptibility to autoimmune lesions, and a higher risk of evolution toward pulmonary hypertension. A better screening for pulmonary hemosiderosis and a better prevention of hypoxia in DS paediatric patients may prevent a severe evolution of the disease.
