Portal de investigação
Diffuse alveolar haemorrhage in children: an international multicentre study
Data de publicação:
Autores da FMUP
Participantes de fora da FMUP
- Ring, AM
- Schwerk, N
- Kiper, N
- Aslan, AT
- Aurora, P
- Ayats, R
- Bandeira, T
- Carlens, J
- Castillo-Corullon, S
- Cobanoglu, N
- Elnazir, B
- Emiralioglu, N
- Eyuboglu, TS
- Fayon, M
- Gursoy, TR
- Hogg, C
- Kotz, K
- Karadag, B
- Latalova, V
- Krenke, K
- Lange, J
- Manali, ED
- Osona, B
- Papiris, S
- Proesmann, M
- Reix, P
- Roditis, L
- Rubak, S
- Rumman, N
- Snijders, D
- Stehling, F
- Weiss, L
- Yalcin, E
- Zirek, F
- Bush, A
- Clement, A
- Griese, M
- Buchvald, FF
- Nathan, N
- Nielsen, KG
Unidades de investigação
Abstract
Background Paediatric diffuse alveolar haemorrhage (DAH) is a rare heterogeneous condition with limited knowledge on clinical presentation, treatment and outcome. Methods A retrospective, descriptive multicentre follow-up study initiated from the European network for translational research in children's and adult interstitial lung disease (Cost Action CA16125) and chILD-EU CRC (the European Research Collaboration for Children's Interstitial Lung Disease). Inclusion criteria were DAH of any cause diagnosed before the age of 18 years. Results Data of 124 patients from 26 centres (15 counties) were submitted, of whom 117 patients fulfilled the inclusion criteria. Diagnoses were idiopathic pulmonary haemosiderosis (n=35), DAH associated with autoimmune features (n=20), systemic and collagen disorders (n=18), immuno-allergic conditions (n=10), other childhood interstitial lung diseases (chILD) (n=5), autoinflammatory diseases (n=3), DAH secondary to other conditions (n=21) and nonspecified DAH (n=5). Median (IQR) age at onset was 5 (2.0-12.9) years. Most frequent clinical presentations were anaemia (87%), haemoptysis (42%), dyspnoea (35%) and cough (32%). Respiratory symptoms were absent in 23%. The most frequent medical treatment was systemic corticosteroids (93%), hydroxychloroquine (35%) and azathioprine (27%). Overall mortality was 13%. Long-term data demonstrated persistent abnormal radiology and a limited improvement in lung function. Conclusions Paediatric DAH is highly heterogeneous regarding underlying causes and clinical presentation. The high mortality rate and number of patients with ongoing treatment years after onset of disease underline that DAH is a severe and often chronic condition. This large international study paves the way for further prospective clinical trials that will in the long term allow evidence-based treatment and follow-up recommendations to be determined.
Dados da publicação
- ISSN/ISSNe:
- 2312-0541, 2312-0541
- Tipo:
- Article
- Páginas:
- 2022-
- Link para outro recurso:
- www.scopus.com
ERJ Open Research European Respiratory Society
Citações Recebidas na Web of Science: 1
Citações Recebidas na Scopus: 6
Documentos
- Não há documentos
Filiações
Keywords
- IDIOPATHIC PULMONARY HEMOSIDEROSIS; INTERSTITIAL LUNG-DISEASE
Financiamento
Proyectos asociados
Hospitalizations for Varicella in children and adolescents in Portugal: 2000 to 2015
Investigador Principal: Maria Inês Ferreira Agueda de Azevedo
Estudo Clínico Académico (Varicella) . 2020
Pulmonary Auscultation using Mobile Devices - Feasibility Study in Respiratory Diseases
Investigador Principal: Maria Inês Ferreira Agueda de Azevedo
Estudo Clínico Académico . 2021
Management of pediatric patients with complicated pneumonia in Portugal, 2000-2015: trends over time and according to regions
Investigador Principal: Maria Inês Ferreira Agueda de Azevedo
Estudo Clínico Académico (Pediatric pneumonia) . 2022
Citar a publicação
Ring AM,Schwerk N,Kiper N,Aslan AT,Aurora P,Ayats R,Azevedo I,Bandeira T,Carlens J,Castillo S,Cobanoglu N,Elnazir B,Emiralioglu N,Eyuboglu TS,Fayon M,Gursoy TR,Hogg C,Kotz K,Karadag B,Latalova V,Krenke K,Lange J,Manali ED,Osona B,Papiris S,Proesmann M,Reix P,Roditis L,Rubak S,Rumman N,Snijders D,Stehling F,Weiss L,Yalcin E,Zirek F,Bush A,Clement A,Griese M,Buchvald FF,Nathan N,Nielsen KG. Diffuse alveolar haemorrhage in children: an international multicentre study. ERJ Open Res. 2023. 9. (2):p. 2022-2022. IF:4,600. (2).
