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One-year outcomes in a multicentre cohort study of incident rare diffuse parenchymal lung disease in children (ChILD)
  1. Steve Cunningham1,2,
  2. Catriona Graham3,
  3. Morag MacLean4,
  4. Paul Aurora5,
  5. Michael Ashworth6,
  6. Angelo Barbato7,
  7. Alistair Calder8,
  8. Julia Carlens9,
  9. Annick Clement10,
  10. Meike Hengst11,
  11. Birgit Kammer12,
  12. Nural Kiper13,
  13. Katarzyna Krenke14,
  14. Kai Kronfeld15,
  15. Joanna Lange14,
  16. Julia Ley-Zaporozhan16,
  17. Andrew G Nicholson17,
  18. Simone Reu18,
  19. Traudl Wesselak11,
  20. Martin Wetzke19,
  21. Andrew Bush20,
  22. Nicolaus Schwerk21,
  23. Matthias Griese22
  24. on behalf of the ChILDEU study group
    1. 1 Centre for Inflammation Research, University of Edinburgh, Edinburgh, UK
    2. 2 Department of Respiratory and Sleep Medicine, Royal Hospital for Sick Children, Edinburgh, UK
    3. 3 Wellcome Trust Clinical Research Facility, Western General Hospital, Edinburgh, UK
    4. 4 Edinburgh Clinical Trials Unit, University of Edinburgh, Edinburgh, UK
    5. 5 Department of Respiratory Paediatrics, Great Ormond Street Hospital, London, UK
    6. 6 Department of Paediatric Pathology, Great Ormond Street Hospital for Children NHS Trust, London, UK
    7. 7 Department of Women’s and Children’s Health, University of Padova, Padua, Italy
    8. 8 Department of Radiology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK
    9. 9 Department of Pediatric Pneumology, Medizinische Hochschule Hannover, Hannover, Germany
    10. 10 Paediatric Pulmonary Department, Trousseau Hospital APHP, Sorbonne Universities and Pierre et Marie Curie University, Paris, France
    11. 11 Pediatric Pneumology, Hauner Children’s University Hospital, Munich, Germany
    12. 12 University Children’s Hospital at Dr. von Haunersches Kinderspital, LMU Munich, Munich, Germany
    13. 13 Pediatric Pulmonology, Hacettepe University, Ankara, Turkey
    14. 14 Department of Pediatric Pneumology and Allergy, Warszawski Uniwersytet Medyczny, Warszawa, Poland
    15. 15 IZKS Mainz, University Medical Center Mainz, Mainz, Germany
    16. 16 Institute for Clinical Radiology, University Hospital of Ludwig Maximilian University, Munich, Germany
    17. 17 Royal Brompton and Harefield NHS Foundation Trust and Imperial College, London, UK
    18. 18 Pathology, Ludwig-Maximilians-Universitat Munchen Medizinische Fakultat, Munchen, Germany
    19. 19 Department of Pediatric Pulmonology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany
    20. 20 Department of Respiratory Paediatrics, Royal Brompton Hospital, London, UK
    21. 21 Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany
    22. 22 Lung Research Group, Children’s Hospital of Ludwig Maximilians University, Munich, Germany
    1. Correspondence to Professor Steve Cunningham, Centre for Inflammation Research, University of Edinburgh, Edinburgh, UK; steve.cunningham{at}nhs.net

    Abstract

    We performed a prospective, observational, cohort study of children newly diagnosed with children’s interstitial lung disease (ChILD), with structured follow-up at 4, 8, 12 weeks and 6 and 12 months. 127 children, median age 0.9 (IQR 0.3–7.9) years had dyspnoea (68%, 69/102), tachypnoea (75%, 77/103) and low oxygen saturation (SpO2) median 92% (IQR 88–96). Death (n=20, 16%) was the most common in those <6 months of age with SpO2<94% and developmental/surfactant disorders. We report for the first time that ChILD survivors improved multiple clinical parameters within 8–12 weeks of diagnosis. These data can inform family discussions and support clinical trial measurements.

    • Interstitial lung disease in Children
    • mortality
    • ventilation
    • oxygen saturation

    http://dx.doi.org/10.1136/thoraxjnl-2019-213217

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      Footnotes

      • Collaborators Dr Jayesh Bhatt, Nottingham Children’s Hospital, UK; Dr Frederik Buchvald, Copenhagen University Hospital, Denmark; Dr Nazan Cobanoglu, Ankara University, Turkey; Dr Fran Child, Royal Children’s Hospital, Manchester, UK; Dr Nwokoro Chinedu, Royal London Hospital, UK; Dr Leonard Donato, Colmar, Civil, Strasbourg, France; Ralph Epaud, INSERM 955 - Equipe 5, Paris, France Dr Ampara Escribano, Valencia, Neumologia Inf, Spain Dr Achim Freihorst, Aalen, Ostalb Klinikum, Germany; Dr Atul Gupta, King’s College Hospital, London, UK; Dr Emma Guy, Leeds General Infirmary, UK; Dr Tobias Hübner, Oldenburg, Zentr. Kind-Jugend, Germany; Dr Petra Kaiser-Labusch, Bremen, Klin. Bremen-Mitte, Germany; Dr Christiane Lex, Göttingen University, Germany; Dr Sarah Mayell, Royal Liverpool Children’s Hospital, UK; Dr Samantha Moss, Royal Victoria Infirmary, Newcastle, UK; Dr Nagehan Emiralioglu, Ankara, Hacettepe University, Turkey; Dr Lutz Nährlich, Giessen, Germany; Dr Nadia Nathan, Hôpital Trousseau, Paris, France; Dr Ruth O’Reilly, Sheffield Children’s Hospital, UK; Dr Nicolas Regamey, Luzern, Kinderspital, Switzerland; Dr Isabelle Rochat, Lausanne, Switzerland; Dr Martin Rosewich, Frankfurt/Main, JWG University, Germany; Dr Tugbu Sismanlar, Ankara, Gazi University, Turkey; Dr Deborah Snijders, Padua University, Italy; Dr Florian Stehling, Essen, University, Germany.

      • Contributors Chief investigator of ChILDEU project: MG. Principle investigator observational study: SC. Developed protocol: SC, CG, MM, ABa, ACle, NK, KKro, AB, NS and MG. Peer review team: PA, MA, ACal, JLZ, AN, SR, TW, AB, NS and MG. Data preparation: JC, MH, KK, KKro, JL and MW. Analysis: SC and CG. Analysis review: AB, NS and MG. Final draft: SC, CG, MM, PA, MA, ABa, ACal, JC, ACle, MH, BK, KKat, NK, KKro, JL, JLZ, AN, SR, TW, MW, AB, NS and MG.

      • Funding The project was funded by the European Union’s Seventh Framework Program under grant agreement n°305653—child-EU.

      • Disclaimer The funders had no role in the writing of the manuscript or the decision to submit it for publication.

      • Competing interests SC and VS report other from Boehringer Ingelheim, outside the submitted work; MG reports other from Vertex and Boehringer Ingelheim, outside the submitted work.

      • Patient consent for publication Not required.

      • Provenance and peer review Not commissioned; externally peer reviewed.

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