Incidence and Prevalence of Children's Diffuse Lung Disease in Spain.

Childhood interstitial lung disease Children Children's Interstitial Lung disease Diffuse lung disease Enfermedad pulmonar intersticial infantil Enfermedades difusas del parénquima pulmonar en pediatría Neumopatías intersticiales en pediatría Niños Paediatrics Pediatría chILD

Journal

Archivos de bronconeumologia

ISSN: 1579-2129

Titre abrégé: Arch Bronconeumol

Pays: Spain

ID NLM: 0354720

Informations de publication

Date de publication:
Jan 2022

Historique:

received: 23 03 2021

revised: 15 05 2021

accepted: 02 06 2021

entrez: 7 3 2022

pubmed: 8 3 2022

medline: 8 3 2022

Statut: ppublish

Résumé

Children's diffuse lung disease, also known as children's Interstitial Lung Diseases (chILD), are a heterogeneous group of rare diseases with relevant morbidity and mortality, which diagnosis and classification are very complex. Epidemiological data are scarce. The aim of this study was to analyse incidence and prevalence of chILD in Spain. Multicentre observational prospective study in patients from 0 to 18 years of age with chILD to analyse its incidence and prevalence in Spain, based on data reported in 2018 and 2019. A total of 381 cases with chILD were notified from 51 paediatric pulmonology units all over Spain, covering the 91.7% of the paediatric population. The average incidence of chILD was 8.18 (CI 95% 6.28-10.48) new cases/million of children per year. The average prevalence of chILD was 46.53 (CI 95% 41.81-51.62) cases/million of children. The age group with the highest prevalence were children under 1 year of age. Different types of disorders were seen in children 2-18 years of age compared with children 0-2 years of age. Most frequent cases were: primary pulmonary interstitial glycogenosis in neonates (17/65), neuroendocrine cell hyperplasia of infancy in infants from 1 to 12 months (44/144), idiopathic pulmonary haemosiderosis in children from 1 to 5 years old (13/74), hypersensitivity pneumonitis in children from 5 to 10 years old (9/51), and scleroderma in older than 10 years old (8/47). We found a higher incidence and prevalence of chILD than previously described probably due to greater understanding and increased clinician awareness of these rare diseases.

Sections du résumé

BACKGROUND BACKGROUND

METHODS METHODS

Multicentre observational prospective study in patients from 0 to 18 years of age with chILD to analyse its incidence and prevalence in Spain, based on data reported in 2018 and 2019.

RESULTS RESULTS

A total of 381 cases with chILD were notified from 51 paediatric pulmonology units all over Spain, covering the 91.7% of the paediatric population. The average incidence of chILD was 8.18 (CI 95% 6.28-10.48) new cases/million of children per year. The average prevalence of chILD was 46.53 (CI 95% 41.81-51.62) cases/million of children. The age group with the highest prevalence were children under 1 year of age. Different types of disorders were seen in children 2-18 years of age compared with children 0-2 years of age. Most frequent cases were: primary pulmonary interstitial glycogenosis in neonates (17/65), neuroendocrine cell hyperplasia of infancy in infants from 1 to 12 months (44/144), idiopathic pulmonary haemosiderosis in children from 1 to 5 years old (13/74), hypersensitivity pneumonitis in children from 5 to 10 years old (9/51), and scleroderma in older than 10 years old (8/47).

CONCLUSIONS CONCLUSIONS

We found a higher incidence and prevalence of chILD than previously described probably due to greater understanding and increased clinician awareness of these rare diseases.

Identifiants

DOI: 10.1016/j.arbres.2021.06.001 PMID: 35249699

pubmed: 35249699

pii: S0300-2896(21)00181-2

doi: 10.1016/j.arbres.2021.06.001

pii:

doi:

Types de publication

Journal Article

Langues

eng spa

Pagination

22-29