Diffuse alveolar haemorrhage in children: an international multicentre study
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 childr...
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| Format: | Article |
| Language: | English |
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European Respiratory Society
2023-04-01
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| Series: | ERJ Open Research |
| Online Access: | http://openres.ersjournals.com/content/9/2/00733-2022.full |
| _version_ | 1827931435813568512 |
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| author | Astrid Madsen Ring Nicolaus Schwerk Nural Kiper Ayse Tana Aslan Paul Aurora Roser Ayats Ines Azevedo Teresa Bandeira Julia Carlens Silvia Castillo-Corullon Nazan Cobanoglu Basil Elnazir Nagehan Emiralioğlu Tugba Sismanlar Eyuboglu Michael Fayon Tugba Ramaslı Gursoy Claire Hogg Karsten Kötz Bülent Karadag Vendula Látalová Katarzyna Krenke Joanna Lange Effrosyni D. Manali Borja Osona Spyros Papiris Marijke Proesmann Philippe Reix Lea Roditis Sune Rubak Nisreen Rumman Deborah Snijders Florian Stehling Laurence Weiss Ebru Yalcın Fazilcan Zirek Andrew Bush Annick Clement Matthias Griese Frederik Fouirnaies Buchvald Nadia Nathan Kim Gjerum Nielsen |
| author_facet | Astrid Madsen Ring Nicolaus Schwerk Nural Kiper Ayse Tana Aslan Paul Aurora Roser Ayats Ines Azevedo Teresa Bandeira Julia Carlens Silvia Castillo-Corullon Nazan Cobanoglu Basil Elnazir Nagehan Emiralioğlu Tugba Sismanlar Eyuboglu Michael Fayon Tugba Ramaslı Gursoy Claire Hogg Karsten Kötz Bülent Karadag Vendula Látalová Katarzyna Krenke Joanna Lange Effrosyni D. Manali Borja Osona Spyros Papiris Marijke Proesmann Philippe Reix Lea Roditis Sune Rubak Nisreen Rumman Deborah Snijders Florian Stehling Laurence Weiss Ebru Yalcın Fazilcan Zirek Andrew Bush Annick Clement Matthias Griese Frederik Fouirnaies Buchvald Nadia Nathan Kim Gjerum Nielsen |
| author_sort | Astrid Madsen Ring |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-13T06:51:29Z |
| format | Article |
| id | doaj.art-583cf26a81df4619a065611a08f4083b |
| institution | Directory Open Access Journal |
| issn | 2312-0541 |
| language | English |
| last_indexed | 2024-03-13T06:51:29Z |
| publishDate | 2023-04-01 |
| publisher | European Respiratory Society |
| record_format | Article |
| series | ERJ Open Research |
| spelling | doaj.art-583cf26a81df4619a065611a08f4083b2023-06-07T13:31:08ZengEuropean Respiratory SocietyERJ Open Research2312-05412023-04-019210.1183/23120541.00733-202200733-2022Diffuse alveolar haemorrhage in children: an international multicentre studyAstrid Madsen Ring0Nicolaus Schwerk1Nural Kiper2Ayse Tana Aslan3Paul Aurora4Roser Ayats5Ines Azevedo6Teresa Bandeira7Julia Carlens8Silvia Castillo-Corullon9Nazan Cobanoglu10Basil Elnazir11Nagehan Emiralioğlu12Tugba Sismanlar Eyuboglu13Michael Fayon14Tugba Ramaslı Gursoy15Claire Hogg16Karsten Kötz17Bülent Karadag18Vendula Látalová19Katarzyna Krenke20Joanna Lange21Effrosyni D. Manali22Borja Osona23Spyros Papiris24Marijke Proesmann25Philippe Reix26Lea Roditis27Sune Rubak28Nisreen Rumman29Deborah Snijders30Florian Stehling31Laurence Weiss32Ebru Yalcın33Fazilcan Zirek34Andrew Bush35Annick Clement36Matthias Griese37Frederik Fouirnaies Buchvald38Nadia Nathan39Kim Gjerum Nielsen40 Paediatric Pulmonary Service, Dept of Paediatrics and Adolescent Medicine, Copenhagen, University Hospital, Rigshospitalet, Copenhagen, Denmark Clinic for Paediatric Pneumology, Allergology and Neonatology, Hannover Medical School, German Center for Lung Research (DZL), Hannover, Germany Hacettepe University Faculty of Medicine, Department of Pediatric Pulmonology, Ankara, Turkey Gazi University Faculty of Medicine, Department of Pediatric Pulmonology, Ankara, Turkey Respiratory Unit, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK Pediatric Pulmonology and Allergology Department, Parc Taulí Hospital Universitari, Institut d'Investigació i Innovació Parc Taulí (I3PT), Universitat Autónoma de Barcelona, Barcelona, Spain Departamento de Ginecologia Obstetrícia e Pediatria, Faculdade de Medicina, Universidade do Porto and Serviço de Pediatria, Centro Hospitalar Universitário de S. João, Porto, Portugal Department of Pediatrics, Respiratory Unit, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Lisbon, Portugal Clinic for Paediatric Pneumology, Allergology and Neonatology, Hannover Medical School, German Center for Lung Research (DZL), Hannover, Germany Pediatric Pulmonology, Clinical Hospital of Valencia, Valencia, Spain Department of Pediatrics, Pediatric Pulmonology Division, Ankara University Faculty of Medicine, Ankara, Turkey CHI at Tallaght University Hospital, Dublin, Ireland Hacettepe University Faculty of Medicine, Department of Pediatric Pulmonology, Ankara, Turkey Gazi University Faculty of Medicine, Department of Pediatric Pulmonology, Ankara, Turkey Pediatric Pulmonology Department, CHU Bordeaux, Bordeaux, France Gazi University Faculty of Medicine, Department of Pediatric Pulmonology, Ankara, Turkey Royal Brompton Hospital, London, UK Devision of Paediatrics, Drottning Silvias Barn- och Ungdomssjukhus, University of Gothenburg, Gothenburg, Sweden Division of Paediatric Pulmonology, Marmara University Faculty of Medicine, Istanbul, Turkey Department of Pediatrics, University Hospital Olomouc, Olomouc, Czech Republic Department of Pediatric Pneumonology and Allergy, Medical University of Warsaw, Warsaw, Poland Department of Pediatric Pneumonology and Allergy, Medical University of Warsaw, Warsaw, Poland 2nd Pulmonary Medicine Department, General University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Athens, Greece Pediatric Pulmonology Department, Son Espases University Hospital, Palma de Mallorca, Spain 2nd Pulmonary Medicine Department, General University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Athens, Greece Pediatric Pulmonology, KUL UZ Gasthuisberg, Leuven, Belgium Université de Lyon, Hôpital Femme Mère Enfant, Pediatric Pulmonology Department, Lyon, France Dept of Pediatric Pulmonology and Allergy, Children's University Hospital, Toulouse, France Danish Center of Pediatric Pulmonology and Allergology, Department of Pediatrics and Adolescents Medicine, University Hospital of Aarhus, Aarhus, Denmark Pediatric Department, Makassed Hospital, East Jerusalem, Palestine Dipartimento Salute della Donna e del Bambino, Università degli Studi di Padova, Padua, Italy Clinic for Paediatrics III, University Hospital Essen, Essen, Germany Strasbourg University, Hôpital de Hautepierre, Pediatric Pulmonology Department, Strasbourg, France Hacettepe University Faculty of Medicine, Department of Pediatric Pulmonology, Ankara, Turkey Department of Pediatrics, Pediatric Pulmonology Division, Ankara University Faculty of Medicine, Ankara, Turkey Imperial College London and Paediatric Respiratory Medicine, Royal Brompton and Harefield NHS Foundation Trust, London, UK Sorbonne Université, Pediatric Pulmonology and Reference Center for rare lung diseases RespiRare, Inserm U933 Laboratory of childhood genetic diseases, Armand Trousseau Hospital, APHP, Paris, France Department of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark Paediatric Pulmonary Service, Dept of Paediatrics and Adolescent Medicine, Copenhagen, University Hospital, Rigshospitalet, Copenhagen, Denmark Sorbonne Université, Pediatric Pulmonology and Reference Center for rare lung diseases RespiRare, Inserm U933 Laboratory of childhood genetic diseases, Armand Trousseau Hospital, APHP, Paris, France Paediatric Pulmonary Service, Dept of Paediatrics and Adolescent Medicine, Copenhagen, University Hospital, Rigshospitalet, Copenhagen, Denmark 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.http://openres.ersjournals.com/content/9/2/00733-2022.full |
| spellingShingle | Astrid Madsen Ring Nicolaus Schwerk Nural Kiper Ayse Tana Aslan Paul Aurora Roser Ayats Ines Azevedo Teresa Bandeira Julia Carlens Silvia Castillo-Corullon Nazan Cobanoglu Basil Elnazir Nagehan Emiralioğlu Tugba Sismanlar Eyuboglu Michael Fayon Tugba Ramaslı Gursoy Claire Hogg Karsten Kötz Bülent Karadag Vendula Látalová Katarzyna Krenke Joanna Lange Effrosyni D. Manali Borja Osona Spyros Papiris Marijke Proesmann Philippe Reix Lea Roditis Sune Rubak Nisreen Rumman Deborah Snijders Florian Stehling Laurence Weiss Ebru Yalcın Fazilcan Zirek Andrew Bush Annick Clement Matthias Griese Frederik Fouirnaies Buchvald Nadia Nathan Kim Gjerum Nielsen Diffuse alveolar haemorrhage in children: an international multicentre study ERJ Open Research |
| title | Diffuse alveolar haemorrhage in children: an international multicentre study |
| title_full | Diffuse alveolar haemorrhage in children: an international multicentre study |
| title_fullStr | Diffuse alveolar haemorrhage in children: an international multicentre study |
| title_full_unstemmed | Diffuse alveolar haemorrhage in children: an international multicentre study |
| title_short | Diffuse alveolar haemorrhage in children: an international multicentre study |
| title_sort | diffuse alveolar haemorrhage in children an international multicentre study |
| url | http://openres.ersjournals.com/content/9/2/00733-2022.full |
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