One-year outcomes in a multicentre cohort study of incident rare diffuse parenchymal lung disease in children (ChILD)

Steve Cunningham; Catriona Graham; Morag MacLean; Paul Aurora; Michael Ashworth; Angelo Barbato; Alistair Calder; Julia Carlens; Annick Clement; Meike Hengst; Birgit Kammer; Nural Kiper; Katarzyna Krenke; Kai Kronfeld; Joanna Lange; Julia Ley-Zaporozhan; Andrew G Nicholson; Simone Reu; Traudl Wesselak; Martin Wetzke; Andrew Bush; Nicolaus Schwerk; Matthias Griese; ChILDEU study group

doi:10.1136/thoraxjnl-2019-213217

One-year outcomes in a multicentre cohort study of incident rare diffuse parenchymal lung disease in children (ChILD)

Thorax. 2020 Feb;75(2):172-175. doi: 10.1136/thoraxjnl-2019-213217. Epub 2019 Nov 20.

Authors

Steve Cunningham^{1

2}, Catriona Graham³, Morag MacLean⁴, Paul Aurora⁵, Michael Ashworth⁶, Angelo Barbato⁷, Alistair Calder⁸, Julia Carlens⁹, Annick Clement¹⁰, Meike Hengst¹¹, Birgit Kammer¹², Nural Kiper¹³, Katarzyna Krenke¹⁴, Kai Kronfeld¹⁵, Joanna Lange¹⁴, Julia Ley-Zaporozhan¹⁶, Andrew G Nicholson¹⁷, Simone Reu¹⁸, Traudl Wesselak¹¹, Martin Wetzke¹⁹, Andrew Bush²⁰, Nicolaus Schwerk²¹, Matthias Griese²²; ChILDEU study group

Affiliations

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

PMID: 31748256
DOI: 10.1136/thoraxjnl-2019-213217

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.

Keywords: Interstitial lung disease in Children; mortality; oxygen saturation; ventilation.

Publication types

Multicenter Study
Observational Study
Research Support, Non-U.S. Gov't

MeSH terms

Adolescent
Adrenal Cortex Hormones / administration & dosage*
Azithromycin / administration & dosage*
Cause of Death
Child
Child, Preschool
Cohort Studies
Drug Therapy, Combination
Europe
Female
Follow-Up Studies
Humans
Hydroxychloroquine / administration & dosage*
Infant
Kaplan-Meier Estimate
Longitudinal Studies
Lung Diseases, Interstitial / diagnosis*
Lung Diseases, Interstitial / drug therapy*
Lung Diseases, Interstitial / epidemiology
Male
Monitoring, Physiologic / methods
Prospective Studies
Registries
Respiratory Function Tests
Risk Assessment
Severity of Illness Index
Survival Analysis
Time Factors

Substances

Adrenal Cortex Hormones
Hydroxychloroquine
Azithromycin