Diffuse alveolar haemorrhage in children : an international multicentre study
Ring, Astrid Madsen (University of Copenhagen)
Schwerk, Nicolaus (German Center for Lung Research (DZL))
Kiper, Nural (Hacettepe University Faculty of Medicine)
Aslan, Ayse Tana (Gazi University Faculty of Medicine)
Aurora, Paul (Great Ormond Street Hospital for Children (Londres))
Ayats-Vidal, Roser (Parc Taulí Hospital Universitari. Institut d'Investigació i Innovació Parc Taulí (I3PT))
Azevedo, Ines (Universidade do Porto and Serviço de Pediatria)
Bandeira, Teresa (Centro Hospitalar Universitário Lisboa Norte)
Carlens, Julia (German Center for Lung Research (DZL))
Castillo-Corullón, Silvia (Hospital Clínic Universitari (València))
Cobanoglu, Nazan (Ankara University Faculty of Medicine)
Elnazir, Basil (CHI at Tallaght University Hospital (Dublin))
Emiralioglu, Nagehan (Hacettepe University Faculty of Medicine)
Eyuboglu, Tugba Sismanlar (Gazi University Faculty of Medicine)
Fayon, Michael (CHU Bordeaux)
Gursoy, Tugba Ramaslı (Gazi University Faculty of Medicine)
Hogg, Claire (Royal Brompton Hospital (Londres))
Kötz, Karsten (University of Gothenburg)
Karadag, Bülent (Marmara University Faculty of Medicine (Turquía))
Látalová, Vendula (University Hospital Olomouc (Olomouc, República Txeca))
Krenke, Katarzyna (Medical University of Warsaw)
Lange, Joanna (Medical University of Warsaw)
Manali, Effrosyni D. (Athens Medical School)
Osona, Borja (Hospital Universitari Son Espases (Palma de Mallorca, Balears))
Papiris, Spyros (2nd National and Kapodistrian University of Athens)
Proesmann, Marijke (KUL UZ Gasthuisberg)
Reix, Philippe (Université de Lyon)
Roditis, Lea (Children's University Hospital (França))
Rubak, Sune (Aarhus University Hospital (Aarhus, Dinamarca))
Rumman, Nisreen (Makassed Hospital)
Snijders, Deborah (Università degli Studi di Padova)
Stehling, Florian (University Hospital Essen (Alemanya))
Weiss, Laurence (Strasbourg University)
Yalcın, Ebru (Hacettepe University Faculty of Medicine)
Zirek, Fazilcan (Ankara University Faculty of Medicine)
Bush, Andrew (Imperial College London and Paediatric Respiratory Medicine)
Clement, Annick (Sorbonne Université)
Griese, Matthias (University of Copenhagen)
Buchvald, Frederik Fouirnaies (University of Copenhagen)
Nathan, Nadia
Nielsen, Kim G
Universitat Autònoma de Barcelona

Data:	2023
Resum:	Paediatric diffuse alveolar haemorrhage (DAH) is a rare heterogeneous condition with limited knowledge on clinical presentation, treatment and outcome. 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. 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. 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. Diffuse alveolar haemorrhage (DAH) in children is a rare condition. DAH is heterogeneous in its underlying cause, presentation and outcome, with high overall mortality. There is a broad variation in diagnostic workup and medical treatment across Europe.
Drets:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, sempre que no sigui amb finalitats comercials, i sempre que es reconegui l'autoria de l'obra original.
Llengua:	Anglès
Document:	Article ; recerca ; Versió publicada
Publicat a:	ERJ Open Research, Vol. 9 (april 2023) , ISSN 2312-0541

DOI: 10.1183/23120541.00733-2022
PMID: 37101741

13 p, 1.6 MB

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