Article Text

Download PDFPDF
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 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. 1Centre for Inflammation Research, University of Edinburgh, Edinburgh, UK
    2. 2Department of Respiratory and Sleep Medicine, Royal Hospital for Sick Children, Edinburgh, UK
    3. 3Wellcome Trust Clinical Research Facility, Western General Hospital, Edinburgh, UK
    4. 4Edinburgh Clinical Trials Unit, University of Edinburgh, Edinburgh, UK
    5. 5Department of Respiratory Paediatrics, Great Ormond Street Hospital, London, UK
    6. 6Department of Paediatric Pathology, Great Ormond Street Hospital for Children NHS Trust, London, UK
    7. 7Department of Women’s and Children’s Health, University of Padova, Padua, Italy
    8. 8Department of Radiology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK
    9. 9Department of Pediatric Pneumology, Medizinische Hochschule Hannover, Hannover, Germany
    10. 10Paediatric Pulmonary Department, Trousseau Hospital APHP, Sorbonne Universities and Pierre et Marie Curie University, Paris, France
    11. 11Pediatric Pneumology, Hauner Children’s University Hospital, Munich, Germany
    12. 12University Children’s Hospital at Dr. von Haunersches Kinderspital, LMU Munich, Munich, Germany
    13. 13Pediatric Pulmonology, Hacettepe University, Ankara, Turkey
    14. 14Department of Pediatric Pneumology and Allergy, Warszawski Uniwersytet Medyczny, Warszawa, Poland
    15. 15IZKS Mainz, University Medical Center Mainz, Mainz, Germany
    16. 16Institute for Clinical Radiology, University Hospital of Ludwig Maximilian University, Munich, Germany
    17. 17Royal Brompton and Harefield NHS Foundation Trust and Imperial College, London, UK
    18. 18Pathology, Ludwig-Maximilians-Universitat Munchen Medizinische Fakultat, Munchen, Germany
    19. 19Department of Pediatric Pulmonology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany
    20. 20Department of Respiratory Paediatrics, Royal Brompton Hospital, London, UK
    21. 21Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany
    22. 22Lung 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
    View Full Text

    Statistics from Altmetric.com

    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.

    Request Permissions

    If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.