MacLean Joanna E, Atenafu Eshetu, Kirby-Allen Melanie, MacLusky Ian B, Stephens Derek, Grasemann Hartmut, Subbarao Padmaja
Department of Pediatrics, Division of Respiratory Medicine, Research Institute, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Am J Respir Crit Care Med. 2008 Nov 15;178(10):1055-9. doi: 10.1164/rccm.200708-1219OC. Epub 2008 Sep 5.
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.
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.
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.
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.
Lung volumes in children with SCD decline with age. The pattern of decline begins in childhood, and supports a predominately restrictive defect.
镰状细胞病（SCD）可导致因肺部并发症引起的显著发病率和死亡率。SCD患儿整个童年期肺功能变化模式尚未得到很好的描述。
确定SCD患儿的肺功能模式是否与种族匹配的预测值在整个童年期有所不同，描述这种变化模式，并研究临床协变量对肺功能的影响。
对来自单一中心的8至18岁SCD患儿的肺功能测量值进行纳入检查。采用混合模型分析回顾性地比较这些患儿的肺功能与种族匹配的参考方程预测值。研究年龄、性别、血红蛋白水平和β-珠蛋白基因型对肺功能纵向变化的影响。
SCD患儿在整个童年期肺活量计测量的肺容积显著下降，这与其他肺容积测量指标的变化模式一致。男性FEV(1)和肺总量的平均下降率分别为预测值的2.93%和2.15%/年，女性分别为2.95%和2.43%/年。已知与更严重疾病相关的β-珠蛋白基因型显示肺功能下降更快，而性别对肺功能的影响不一致。
SCD患儿的肺容积随年龄下降。这种下降模式始于儿童期，支持主要为限制性缺陷。
