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Diffuse alveolar ha...
Diffuse alveolar haemorrhage in children: an international multicentre study
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Ring, A. M. (author)
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Schwerk, N. (author)
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Kiper, N. (author)
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show more...
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Aslan, A. T. (author)
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Aurora, P. (author)
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Ayats, R. (author)
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Azevedo, I. (author)
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Bandeira, T. (author)
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Carlens, J. (author)
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Castillo-Corullon, S. (author)
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Cobanoglu, N. (author)
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Elnazir, B. (author)
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Emiralioglu, N. (author)
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Eyuboglu, T. S. (author)
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Fayon, M. (author)
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Gursoy, T. R. (author)
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Hogg, C. (author)
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- Kötz, Karsten (author)
- Gothenburg University,Göteborgs universitet,Institutionen för kliniska vetenskaper, Avdelningen för pediatrik,Institute of Clinical Sciences, Department of Pediatrics
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Karadag, B. (author)
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Latalova, V. (author)
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Krenke, K. (author)
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Lange, J. (author)
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Manali, E. D. (author)
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Osona, B. (author)
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Papiris, S. (author)
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Proesmann, M. (author)
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Reix, P. (author)
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Roditis, L. (author)
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Rubak, S. (author)
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Rumman, N. (author)
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Snijders, D. (author)
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Stehling, F. (author)
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Weiss, L. (author)
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Yalcin, E. (author)
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Zirek, F. (author)
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Bush, A. (author)
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Clement, A. (author)
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Griese, M. (author)
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Buchvald, F. F. (author)
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Nathan, N. (author)
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Nielsen, K. G. (author)
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(creator_code:org_t)
- 2023
- 2023
- English.
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In: ERJ Open Research. ; 9:2, s. 00733-2022
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https://gup.ub.gu.se...
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https://doi.org/10.1...
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Abstract
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- 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.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Lungmedicin och allergi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Respiratory Medicine and Allergy (hsv//eng)
Keyword
- idiopathic pulmonary hemosiderosis
- interstitial lung-disease
- Respiratory System
Publication and Content Type
- ref (subject category)
- art (subject category)
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- By the author/editor
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Ring, A. M.
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Schwerk, N.
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Kiper, N.
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Aslan, A. T.
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Aurora, P.
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Ayats, R.
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show more...
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Azevedo, I.
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Bandeira, T.
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Carlens, J.
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Castillo-Corullo ...
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Cobanoglu, N.
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Elnazir, B.
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Emiralioglu, N.
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Eyuboglu, T. S.
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Fayon, M.
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Gursoy, T. R.
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Hogg, C.
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Kötz, Karsten
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Karadag, B.
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Latalova, V.
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Krenke, K.
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Lange, J.
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Manali, E. D.
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Osona, B.
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Papiris, S.
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Proesmann, M.
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Reix, P.
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Roditis, L.
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Rubak, S.
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Rumman, N.
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Snijders, D.
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Stehling, F.
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Weiss, L.
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Yalcin, E.
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Zirek, F.
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Bush, A.
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Clement, A.
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Griese, M.
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Buchvald, F. F.
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Nathan, N.
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Nielsen, K. G.
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show less...
- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Respiratory Medi ...
- Articles in the publication
- ERJ Open Researc ...
- By the university
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University of Gothenburg