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Diffuse alveolar haemorrhage in children: an international multicentre study
AM. Ring, N. Schwerk, N. Kiper, AT. Aslan, P. Aurora, R. Ayats, I. Azevedo, T. Bandeira, J. Carlens, S. Castillo-Corullon, N. Cobanoglu, B. Elnazir, N. Emiralioğlu, TS. Eyuboglu, M. Fayon, TR. Gursoy, C. Hogg, K. Kötz, B. Karadag, V. Látalová, K....
Status neindexováno Jazyk angličtina Země Anglie, Velká Británie
Typ dokumentu časopisecké články
NLK
Directory of Open Access Journals
od 2015
PubMed Central
od 2015
Europe PubMed Central
od 2015
Open Access Digital Library
od 2015-01-01
ROAD: Directory of Open Access Scholarly Resources
od 2015
- Publikační typ
- časopisecké články MeSH
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.
CHI at Tallaght University Hospital Dublin Ireland
Clinic for Paediatrics 3 University Hospital Essen Essen Germany
Department of Clinical Medicine University of Copenhagen Copenhagen Denmark
Department of Pediatric Pneumonology and Allergy Medical University of Warsaw Warsaw Poland
Department of Pediatrics University Hospital Olomouc Olomouc Czech Republic
Dept of Pediatric Pulmonology and Allergy Children's University Hospital Toulouse France
Dipartimento Salute della Donna e del Bambino Università degli Studi di Padova Padua Italy
Division of Paediatric Pulmonology Marmara University Faculty of Medicine Istanbul Turkey
Gazi University Faculty of Medicine Department of Pediatric Pulmonology Ankara Turkey
Hacettepe University Faculty of Medicine Department of Pediatric Pulmonology Ankara Turkey
Pediatric Department Makassed Hospital East Jerusalem Palestine
Pediatric Pulmonology Clinical Hospital of Valencia Valencia Spain
Pediatric Pulmonology Department CHU Bordeaux Bordeaux France
Pediatric Pulmonology Department Son Espases University Hospital Palma de Mallorca Spain
Pediatric Pulmonology KUL UZ Gasthuisberg Leuven Belgium
Respiratory Unit Great Ormond Street Hospital for Children NHS Foundation Trust London UK
Royal Brompton Hospital London UK
Strasbourg University Hôpital de Hautepierre Pediatric Pulmonology Department Strasbourg France
These authors contributed equally
Université de Lyon Hôpital Femme Mère Enfant Pediatric Pulmonology Department Lyon France
Citace poskytuje Crossref.org
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