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Vol. 58. Issue 1.
Pages 22-29 (January 2022)
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Vol. 58. Issue 1.
Pages 22-29 (January 2022)
Original Article
Incidence and Prevalence of Children's Diffuse Lung Disease in Spain
Incidencia y prevalencia de las neumopatías intersticiales pediátricas en España
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Alba Torrent-Vernettaa,b,c,d, Mirella Gabolie, Silvia Castillo-Corullónf, Pedro Mondéjar-Lópezg, Verónica Sanz Santiagoh, Jordi Costa-Colomeri, Borja Osonaj, Javier Torres-Borregok, Olga de la Serna-Blázquezl, Sara Bellón Alonsom, Pilar Caro Aguileran, Álvaro Gimeno-Díaz de Ataurio, Alfredo Valenzuela Soriap, Roser Ayatsq, Carlos Martin de Vicenter, Valle Velasco Gonzálezs, José Domingo Moure Gonzálezt, Elisa María Canino Calderínu, María Dolores Pastor-Viverov, María Ángeles Villar Álvarezw, Sandra Rovira-Amigoa,b,c,d, Ignacio Iglesias Serranoa,b,c, Ana Díez Izquierdoa,b,c, Inés de Mir Messaa,b,c, Silvia Gartnera,b,c, Alexandra Navarrox, Noelia Baz-Redónb,c, Rosario Carmonay, Núria Camats-Tarruellab,c,d, Mónica Fernández-Canciob,c,d, Christina Rappz, Joaquin Dopazoy,aa, Matthias Griesez, Antonio Moreno-Galdóa,b,c,d,1,
Corresponding author
amoreno@vhebron.net

Corresponding author.
, on behalf of ChILD-Spain Group 2
a Paediatric Allergy and Pulmonology Section, Department of Paediatrics, Vall d’Hebron Hospital Universitari, Vall d’Hebron Barcelona Hospital Campus, Universitat Autònoma de Barcelona, Spain
b Vall d’Hebron Institut de Recerca (VHIR), Hospital Universitari Vall d’Hebron, Barcelona, Spain
c Department of Paediatrics, Obstetrics, Gynecology, Preventative Medicine and Public Health, Universitat Autònoma de Barcelona, Bellaterra, Barcelona, Spain
d Center for Biomedical Research on Rare Diseases (CIBERER), Instituto de Salud Carlos III, Spain
e Paediatric Allergy and Pulmonology Section, Department of Paediatrics, University Hospital Virgen del Rocío, Sevilla, Spain
f Paediatric Pulmonology and Cystic Fibrosis Department, Hospital Clínico Universitario de Valencia, Spain
g Paediatric Pulmonology and Cystic Fibrosis Unit, University Clinic Virgen de la Arrixaca Hospital, University of Murcia, Spain
h Paediatric Pulmonology and Cystic Fibrosis Department, Hospital Universitario Infantil Niño Jesús, Madrid, Spain
i Paediatric Pulmonology and Cystic Fibrosis Department, Hospital Sant Joan de Déu, Barcelona, Spain
j Paediatric Pulmonology and Allergology Department, Hospital Universitario Son Espases, Palma de Mallorca, Spain
k Hospital Universitario Reina Sofía, Córdoba, Spain
l Paediatric Pulmonology Department, Hospital Universitario La Paz, Madrid, Spain
m Paediatric Pulmonology Department, Paediatrics Department, Hospital Universitario Gregorio Marañón, Madrid, Spain
n Paediatric Pulmonology Department, Hospital Regional Universitario de Málaga, IBIMA, Spain
o Paediatric Pulmonology and Allergology Department, 12 de Octubre University Hospital, Madrid, Spain
p Paediatric Pulmonology Department, Hospital Universitario Virgen de las Nieves, Granada, Spain
q Paediatric Pulmonology and Allergology Department, Parc Taulí Hospital Universitari, Institut d’Investigació i Innovació Parc Taulí I3PT, Sabadell, Universitat Autònoma de Barcelona, Barcelona, Spain
r Paediatric Pulmonology Department, Hospital Universitario Miguel Servet, Zaragoza, Spain
s Paediatric Pulmonology Department, Hospital Universitario de Canarias, Tenerife, Spain
t Paediatric Pulmonology and Allergology Department, Paediatrics Department, Hospital Clínico Universitario de Santiago, Spain
u Paediatric Pulmonology Department, Hospital Universitario Materno Infantil Canarias, Las Palmas de Gran Canaria, Spain
v Paediatric Pulmonology Department, Hospital Universitario Cruces, IIS Biocruces Bizkaia, Barakaldo, Bizkaia, Spain
w Paediatric Pulmonology Department, Hospital Universitario Basurto, Bilbao, Spain
x Pathology Department, Vall d’Hebron Hospital Universitari, Vall d’Hebron Barcelona Hospital Campus, Universitat Autònoma de Barcelona, Spain
y Clinical Bioinformatics Area, Fundación Progreso y Salud (FPS), CDCA, University Hospital Virgen del Rocío, Sevilla, Spain
z Division of Pediatric Pneumology, Dr. von Hauner Children's Hospital, University Hospital Munich, Germany
aa Computational Systems Medicine, Institute of Biomedicine of Seville (IBIS), Bioinformatics in Rare Diseases (BiER), Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), FPS, Functional Genomics Node (INB), FPS, University Hospital Virgen del Rocío, Sevilla, Spain
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Figures (2)
Tables (4)
Table 1. Incidence and Prevalence Each Year of the Study Period.
Table 2. Prevalence Data of the Transversal Study in October 2019, Stratified by Age of Patients at Initial Symptoms.
Table 3. Frequency and Classification of Prevalent chILD Cases.
Table 4. Disorders With an Identified Gene Mutation Which Lead to the Diagnosis of chILD.
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Additional material (1)
Abstract
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.

Keywords:
Children's Interstitial Lung disease
Diffuse lung disease
chILD
Paediatrics
Children
Childhood interstitial lung disease
Resumen
Antecedentes

Las neumopatías intersticiales pediátricas, también conocidas con el acrónimo chILD (del inglés children's Interstitial Lung Diseases), es un grupo heterogéneo de enfermedades raras con morbimortalidad relevante, cuyo diagnóstico y clasificación son complejos. Los estudios epidemiológicos son escasos. El objetivo de este trabajo fue analizar la incidencia y la prevalencia de chILD en España.

Métodos

Estudio prospectivo observacional multicéntrico en pacientes de 0 a 18 años afectos de chILD para analizar la incidencia y la prevalencia en España, a partir de datos recogidos en 2018 y 2019.

Resultados

Se recogieron 381 casos de chILD entre 51 unidades de neumología pediátrica de toda España, que cubrían el 91,7% de la población pediátrica. La incidencia promedio fue 8,18 (IC 95%: 6,28-10,48) casos nuevos/millón de niños por año. La prevalencia promedio fue de 46,53 (IC 95%: 41,81-51,62) casos/millón de niños. El grupo de edad con mayor prevalencia fue el de niños menores de un año. Se observaron diferentes entidades en niños de 2 a 18 años en comparación con niños de 0 a 2 años. Los diagnósticos más frecuentes fueron: glucogenosis intersticial pulmonar primaria en neonatos (17/65), hiperplasia de células neuroendocrinas en lactantes de uno a 12 meses (44/144), hemosiderosis pulmonar idiopática en niños de uno a 5 años (13/74), neumonía por hipersensibilidad en niños de 5 a 10 años (9/51) y esclerodermia en mayores de 10 años (8/47).

Conclusiones

Encontramos una mayor incidencia y prevalencia de chILD que las descritas previamente, probablemente debido a un mayor conocimiento y detección de estas enfermedades raras.

Palabras clave:
Neumopatías intersticiales en pediatría
Enfermedades difusas del parénquima pulmonar en pediatría
chILD
Pediatría
Niños
Enfermedad pulmonar intersticial infantil

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