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Heterogeneity of respiratory disease in children and young adults with sickle cell disease
  1. Alan Lunt1,
  2. Lucy Mortimer1,
  3. David Rees2,
  4. Sue Height2,
  5. Swee Lay Thein2,3,
  6. Anne Greenough1,4
  1. 1Division of Asthma, Allergy and Lung Biology, MRC & Asthma UK Centre in Allergic Mechanisms of Asthma, King’s College London, London, UK
  2. 2Department of Paediatric Haematology, King’s College Hospital NHS Foundation Trust, London, UK
  3. 3Molecular Haematology, Division of Cancer Studies, King’s College London, London, UK
  4. 4NIHR Biomedical Research Centre at Guy’s and St Thomas’ NHS Foundation Trust and King’s College London, London, UK
  1. Correspondence to Professor Anne Greenough, Neonatal Intensive Care Unit, King’s College Hospital, Denmark Hill, London SE5 9RS, UK; anne.greenough{at}


To detect and characterise different phenotypes of respiratory disease in children and young adults with sickle cell disease (SCD), 11 lung function and haematological biomarkers were analysed using k-means cluster analysis in a cohort of 114 subjects with SCD aged between 5 and 27 years. Three clusters were detected: cluster 1 had elevated pulmonary capillary blood volume, mixed obstructive/restrictive lung disease, hypoxia and moderately severe anaemia; cluster 2 were older patients with restrictive lung disease; and cluster 3 were younger patients with obstructive lung disease, elevated serum lactate dehydrogenase and bronchodilator reversibility. These results may inform more personalised management strategies to improve outcomes.

  • respiratory measurement
  • lung physiology
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  • Contributors AL and AG designed the study. AL, LM, DR and SH collected the data. AL analysed the data. All authors were involved in the development of the manuscript and approved the final version.

  • Funding The research was supported by the National Institute for Health Research (NIHR) Biomedical Research Centre based at Guy’s and St Thomas' NHS Foundation Trust and King’s College London.

  • Disclaimer The views expressed are those of the author(s) and not necessarily those of the NHS, the NIHR or the Department of Health.

  • Competing interests None declared.

  • Ethics approval The studies were approved by King’s College Hospital Research Ethics Committee.

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

  • Data sharing statement The data can be open for sharing if required.

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