Heterogeneity of respiratory disease in children and young adults with sickle cell disease. Issue 6 (29th November 2017)

• Record Type: Journal Article
• Authors: Lunt, Alan; Mortimer, Lucy; Rees, David; Height, Sue; Thein, Swee Lay; Greenough, Anne
• Is Part Of: Thorax. Volume 73:Issue 6(2018)
• Page Start: 575
• Page End: 577
• Publication Date: 2017-11-29
• Subjects: respiratory measurement -- lung physiology; Chest -- Diseases -- Periodicals; Thorax; Chest -- Diseases; Periodicals; Periodicals; 617.54
• DOI: 10.1136/thoraxjnl-2017-210206
• Languages: English
• ISSNs: 0040-6376

Abstract

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.