TY - JOUR
T1 - Incidence and Prevalence of Children's Diffuse Lung Disease in Spain
AU - Torrent-Vernetta, Alba
AU - Gaboli, Mirella
AU - Castillo-Corullón, Silvia
AU - Mondéjar-López, Pedro
AU - Sanz Santiago, Verónica
AU - Costa-Colomer, Jordi
AU - Osona, Borja
AU - Torres-Borrego, Javier
AU - de la Serna-Blázquez, Olga
AU - Bellón Alonso, Sara
AU - Caro Aguilera, Pilar
AU - Gimeno-Díaz de Atauri, Álvaro
AU - Valenzuela Soria, Alfredo
AU - Ayats, Roser
AU - Martin de Vicente, Carlos
AU - Velasco González, Valle
AU - Moure González, José Domingo
AU - Canino Calderín, Elisa María
AU - Pastor-Vivero, María Dolores
AU - Villar Álvarez, María Ángeles
AU - Rovira-Amigo, Sandra
AU - Iglesias Serrano, Ignacio
AU - Díez Izquierdo, Ana
AU - de Mir Messa, Inés
AU - Gartner, Silvia
AU - Navarro, Alexandra
AU - Baz-Redón, Noelia
AU - Carmona, Rosario
AU - Camats-Tarruella, Núria
AU - Fernández-Cancio, Mónica
AU - Rapp, Christina
AU - Dopazo, Joaquin
AU - Griese, Matthias
AU - Moreno-Galdó, Antonio
N1 - Copyright © 2021 SEPAR. Publicado por Elsevier España, S.L.U. All rights reserved.
PY - 2022/1
Y1 - 2022/1
N2 - BACKGROUND: 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.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: 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: 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.
AB - BACKGROUND: 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.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: 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: 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.
KW - Children's Interstitial Lung disease
KW - Diffuse lung disease
KW - chILD
KW - Children
KW - Childhood interstitial lung disease
U2 - 10.1016/j.arbres.2021.06.001
DO - 10.1016/j.arbres.2021.06.001
M3 - Article
C2 - 35249699
SN - 0300-2896
VL - 58
SP - 22
EP - 29
JO - Archivos de bronconeumologia
JF - Archivos de bronconeumologia
IS - 1
ER -