Heterogeneity of respiratory disease in children and young adults with sickle cell disease

TY - JOUR

T1 - Heterogeneity of respiratory disease in children and young adults with sickle cell disease

AU - Lunt, Alan

AU - Mortimer, Lucy

AU - Rees, David

AU - Height, Sue

AU - Thein, Swee Lay

AU - Greenough, Anne

N1 - © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

PY - 2018

Y1 - 2018

N2 - 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.

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.

U2 - 10.1136/thoraxjnl-2017-210206

DO - 10.1136/thoraxjnl-2017-210206

M3 - Article

C2 - 29187592

SN - 0040-6376

VL - 73

SP - 575

EP - 577

JO - Thorax

JF - Thorax

ER -