Science and Research

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

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.
  • Cunningham, S.
  • Graham, C.
  • MacLean, M.
  • Aurora, P.
  • Ashworth, M.
  • Barbato, A.
  • Calder, A.
  • Carlens, J.
  • Clement, A.
  • Hengst, M.
  • Kammer, B.
  • Kiper, N.
  • Krenke, K.
  • Kronfeld, K.
  • Lange, J.
  • Ley-Zaporozhan, J.
  • Nicholson, A. G.
  • Reu, S.
  • Wesselak, T.
  • Wetzke, M.
  • Bush, A.
  • Schwerk, N.
  • Griese, M.
  • Ch, Ildeu study group

Keywords

  • 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/*drug therapy/epidemiology
  • Male
  • Monitoring, Physiologic/methods
  • Prospective Studies
  • Registries
  • Respiratory Function Tests
  • Risk Assessment
  • Severity of Illness Index
  • Survival Analysis
  • Time Factors
  • *Interstitial lung disease in Children
  • *mortality
  • *oxygen saturation
  • *ventilation
  • the submitted work
  • MG reports other from Vertex and Boehringer Ingelheim,
  • outside the submitted work.
Publication details
DOI: 10.1136/thoraxjnl-2019-213217
Journal: Thorax
Pages: 172-175 
Number: 2
Work Type: Original
Location: BREATH
Disease Area: DPLD
Partner / Member: MHH
Access-Number: 31748256
See publication on PubMed

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