Longitudinal decline in lung volume in a population of children with sickle cell disease.
BACKGROUND
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.


OBJECTIVE
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 beta-globin genotype on longitudinal changes in lung function was examined.


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 FEV(1) and total lung capacity is 2.93 and 2.15% predicted/year for males and 2.95 and 2.43% predicted/year for females. beta-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.
MacLean
Joanna E
JE
Department of Pediatrics, Division of Respiratory Medicine, Research Institute, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. joannam4@med.usyd.edu.au
Atenafu
Eshetu
E
Kirby-Allen
Melanie
M
MacLusky
Ian B
IB
Stephens
Derek
D
Grasemann
Hartmut
H
Subbarao
Padmaja
P
D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000235:genetics / Q000503:physiopathology*; D002648:Child; D005260:Female; D005541:Forced Expiratory Volume; D020022:Genetic Predisposition to Disease; D005838:Genotype; D006445:Hemoglobin C Disease / Q000503:physiopathology*; D006451:Hemoglobin, Sickle / Q000235:genetics; D006801:Humans; D008137:Longitudinal Studies; D008168:Lung / Q000503:physiopathology*; D008297:Male; D012189:Retrospective Studies; D014109:Total Lung Capacity
D006451:Hemoglobin, Sickle