RT Journal Article
SR Electronic
T1 Heterogeneity of respiratory disease in children and young adults with sickle cell disease
JF Thorax
JO Thorax
FD BMJ Publishing Group Ltd and British Thoracic Society
SP 575
OP 577
DO 10.1136/thoraxjnl-2017-210206
VO 73
IS 6
A1 Lunt, Alan
A1 Mortimer, Lucy
A1 Rees, David
A1 Height, Sue
A1 Thein, Swee Lay
A1 Greenough, Anne
YR 2018
UL http://thorax.bmj.com/content/73/6/575.abstract
AB 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.