Field Joshua J, DeBaun Michael R, Yan Yan, Strunk Robert C
Department of Internal Medicine, Washington University School of Medicine, St. Louis, Missouri.
Departments of Pediatrics, Biostatistics and Neurology, Washington University School of Medicine, St. Louis, Missouri.
Pediatr Pulmonol. 2008 Nov;43(11):1061-1066. doi: 10.1002/ppul.20883.
Lung disease is a common cause of morbidity among children with sickle cell disease (SCD). Although cross-sectional studies of children with SCD describe abnormal pulmonary function, the pattern of lung function growth in these children compared to children in the general population is not known. To provide preliminary evidence that growth of lung function is attenuated in children with SCD, we conducted a retrospective cohort study of children with hemoglobin SS (HbSS) ages 6-19 years who received at least two spirometry assessments for clinical care. The growth of lung function in these cases was compared to age, gender, and race-specific children without SCD or respiratory complaints from the Harvard Six Cities Study (H6CS). Seventy-nine children with HbSS contributed 363 spirometry measurements (mean per child = 4.6, median = 4.0, range = 2-17) and 255 controls contributed 1,543 spirometry measurements (mean per child = 6.1, median = 6.0, range = 2-13). Longitudinal forced expiratory volume in 1 sec (FEV(1)) was lower for boys and girls with HbSS compared to children in the general population, P = 0.031 and P = 0.002, respectively. When compared to the H6CS cohort, girls with HbSS showed lower longitudinal forced vital capacity (FVC) (P < 0.001) and FEV(1)/FVC (0.038); there was no difference in FVC or FEV(1)/FVC between boys in the HbSS and H6CS cohort. We conclude that growth of lung function is reduced in children with HbSS compared to children in the general population. Gender may influence the risk of developing abnormal lung function and airway obstruction in children with HbSS.
肺部疾病是镰状细胞病（SCD）患儿发病的常见原因。尽管对SCD患儿的横断面研究描述了其肺功能异常，但与普通人群中的儿童相比，这些患儿肺功能的生长模式尚不清楚。为了提供初步证据证明SCD患儿的肺功能生长受到抑制，我们对6至19岁的血红蛋白SS（HbSS）患儿进行了一项回顾性队列研究，这些患儿因临床护理接受了至少两次肺活量测定评估。将这些病例的肺功能生长情况与来自哈佛六城市研究（H6CS）的无SCD或呼吸道疾病主诉的年龄、性别和种族特异性儿童进行比较。79名HbSS患儿提供了363次肺活量测定数据（平均每名患儿4.6次，中位数4.0次，范围2 - 17次），255名对照儿童提供了1543次肺活量测定数据（平均每名患儿6.1次，中位数6.0次，范围2 - 13次）。与普通人群中的儿童相比，HbSS患儿男孩和女孩的1秒用力呼气容积（FEV(1)）纵向值均较低，P值分别为0.031和0.002。与H6CS队列相比，HbSS患儿女孩的用力肺活量（FVC）纵向值较低（P < 0.001），FEV(1)/FVC为0.038；HbSS队列男孩与H6CS队列男孩的FVC或FEV(1)/FVC无差异。我们得出结论，与普通人群中的儿童相比，HbSS患儿的肺功能生长降低。性别可能影响HbSS患儿出现异常肺功能和气道阻塞的风险。
