Progression and Prognostic Indicators of Bronchial Disease in Children with Sickle Cell Disease

Purpose The pulmonary complications of sickle cell disease (SCD) are a leading cause of morbidity and mortality (MacLean et al. Am J Respir Crit Care Med 178:1055–1059, 2008; Klings et al. Am J Respir Crit Care Med 173:1264–1269, 2006; National Heart, Lung, and Blood Institute, 2009). Despite this recognition, predictive markers of lung dysfunction progression remain elusive (Klings et al. Am J Respir Crit Care Med 173:1264–1269, 2006; Platt et al. N Engl J Med 330:1639–1644, 1994; Caboot et al. Curr Opin Pediatr 20:279–287, 2008; Field et al. Am J Hematol 83:574–576, 2008; Shirlo et al. Peadiatr Respir Review 12:78–82, 2011). This study was designed describe the longitudinal progression and identify specific markers that influence bronchial disease in SCD.