Heterogeneity of respiratory disease in children and young adults with sickle cell disease
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Authors
Lunt, Alan
Mortimer, Lucy
Rees, David
Height, Sue
Thein, Swee Lay
Greenough, Anne
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Issue Date
2018
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Article
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Abstract
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.
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Journal
Thorax
Volume
73
Issue
6