title: Longitudinal decline in lung volume in a population of children with sickle cell disease.
abstract: 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.
authors.affiliation: Department of Pediatrics, Division of Respiratory Medicine, Research Institute, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. joannam4@med.usyd.edu.au
authors.forename: Joanna E
authors.initials: JE
authors.lastname: MacLean
authors.forename: Eshetu
authors.initials: E
authors.lastname: Atenafu
authors.forename: Melanie
authors.initials: M
authors.lastname: Kirby-Allen
authors.forename: Ian B
authors.initials: IB
authors.lastname: MacLusky
authors.forename: Derek
authors.initials: D
authors.lastname: Stephens
authors.forename: Hartmut
authors.initials: H
authors.lastname: Grasemann
authors.forename: Padmaja
authors.initials: P
authors.lastname: Subbarao
mesh_terms: 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
chemical_list: D006451:Hemoglobin, Sickle