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Longitudinal Decline in Lung Volume in a Population of Children with Sickle Cell Disease

¹ Division of Respiratory Medicine, ² Child Health Evaluative Sciences, Research Institute, ³ Division of Hematology/Oncology, Department of Pediatrics, the Hospital for Sick Children and the University of Toronto, Toronto, Ontario, Canada

Correspondence and requests for reprint should be addressed to Joanna E. MacLean, M.D., F.A.A.P., F.R.C.P (C), Department of Respiratory Medicine, Children's Hospital at Westmead, Locked Bag 4001, Westmead, New South Wales 2145, Australia. E-mail: joannam4{at}med.usyd.edu.au

Rationale: Sickle cell disease (SCD) results in significant morbidity and mortality attributable to pulmonary complications. The pattern of lung function change across childhood in SCD is not well delineated.

Objectives: To determine if the pattern of lung function in SCD differs from race-matched, predicted values across childhood, to describe that pattern of change, and to examine the effect of clinical covariates on lung function.

Methods: Lung function measurements for children with SCD, aged 8–18 years, from a single center were examined for inclusion. Mixed-model analysis was used to retrospectively review lung function in these children in comparison with those predicted by race-matched reference equations. The contribution of age, sex, Hb level, and β-globin genotype on longitudinal changes in lung function was examined.

Measurements and Main Results: Children with SCD show significant decline in spirometric lung volumes across childhood that are concordant with the pattern of change in other measures of lung volume. The average decline for FEV₁ and total lung capacity is 2.93 and 2.15% predicted/year for males and 2.95 and 2.43% predicted/year for females. β-Globin genotypes known to be associated with more severe disease showed a faster decline in lung function, whereas sex showed an inconsistent effect on lung function.

Conclusions: Lung volumes in children with SCD decline with age. The pattern of decline begins in childhood, and supports a predominately restrictive defect.

Key Words: spirometry • lung volume • genotype • race matched

AT A GLANCE COMMENTARY Scientific Knowledge on the Subject Sickle cell disease (SCD) is a common genetic disease with significant morbidity and mortality due to progressive lung disease. Although patients with SCD have been reported to have both obstructive and restrictive changes, changes in the pattern of lung function over time from childhood are not well delineated. What This Study Adds to the Field Lung volume, as a percentage of that predicted, declines with age in children with SCD; this decline begins in childhood. The rate of decline is similar to that of children with cystic fibrosis and greater than that of children with asthma.

 

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				A. C. Koumbourlis, D. J. Lee, and A. Lee Changes in Lung Function in Children with Sickle Cell Disease Am. J. Respir. Crit. Care Med., August 15, 2009; 180(4): 377 - 377. [Full Text] [PDF]

				J. E. MacLean, H. Grasemann, and P. Subbarao Changes in Lung Function in Children with Sickle Cell Disease Am. J. Respir. Crit. Care Med., August 15, 2009; 180(4): 377 - 378. [Full Text] [PDF]

				A. Bush Update in Pediatric Lung Disease 2008 Am. J. Respir. Crit. Care Med., April 15, 2009; 179(8): 637 - 649. [Full Text] [PDF]