Koumbourlis Anastassios C, Lee Donna J, Lee Ada
Division of Pediatric Pulmonology, Morgan Stanley Children's Hospital of New York, Columbia University Medical Center, New York, New York, USA.
Pediatr Pulmonol. 2008 Feb;43(2):175-8. doi: 10.1002/ppul.20752.
To investigate the changes in lung function and somatic growth that occur over time in children with hemoglobin SC (Hb-SC) sickle cell disease (SCD).
Measurements of lung function and somatic growth were performed in patients with Hb-SC twice with an interval of 50.2 +/- 26.0 months. Comparisons were made with a group of patients with hemoglobin SS (Hb-SS) SCD, matched by age, race, and gender who underwent similar testing and served as controls.
Indices of lung function in patients with Hb-SC were and remained within the normal range in the two testing periods and they were significantly higher than those measured among patients with Hb-SS. However, there was significant and similar decline (as percentage from baseline) over time in both groups (forced vital capacity, FVC: -3.7 +/- 9.4 vs. -3.8 +/- 14.2; forced expiratory volume in the first second, FEV1: -7.4 +/- 9.3 vs. -6.8 +/- 15.2; forced expiratory flow at 25-75% of FVC, FEF(25-75): -13.7 +/- 20.6 vs. -12.1 +/- 24.7 for Hb-SC and Hb-SS respectively). The body mass index (BMI, percentile) was significantly (P < 0.05) higher among patients with Hb-SC (49 +/- 36 vs. 26 +/- 18) and increased over time in both groups (50 +/- 33 vs. 32 +/- 31).
Lung function is generally normal among children with Hb-SC, but it declines over time in a fashion similar to that observed among patients with Hb-SS SCD. The decline is slow and it is not associated with changes in somatic growth. Our findings suggest that patients with Hb-SC should probably have the same close follow-up as patients with Hb-SS.
研究血红蛋白SC（Hb-SC）型镰状细胞病（SCD）患儿肺功能和身体生长随时间的变化。
对Hb-SC患者进行两次肺功能和身体生长测量，间隔时间为50.2±26.0个月。与一组年龄、种族和性别匹配的血红蛋白SS（Hb-SS）型SCD患者进行比较，这些患者接受了类似测试并作为对照。
Hb-SC患者的肺功能指标在两个测试期均处于正常范围内，且显著高于Hb-SS患者测得的指标。然而，两组随时间均出现显著且相似的下降（相对于基线的百分比）（用力肺活量，FVC：-3.7±9.4 vs. -3.8±14.2；第1秒用力呼气量，FEV1：-7.4±9.3 vs. -6.8±15.2；FVC 25%-75%时的用力呼气流量，FEF(25-75)：Hb-SC和Hb-SS分别为-13.7±20.6 vs. -12.1±24.7）。Hb-SC患者的体重指数（BMI，百分位数）显著更高（P<0.05）（49±36 vs. 26±18），且两组均随时间增加（50±33 vs. 32±31）。
Hb-SC患儿的肺功能一般正常，但随时间下降的方式与Hb-SS型SCD患者相似。下降缓慢，且与身体生长变化无关。我们的研究结果表明，Hb-SC患者可能应与Hb-SS患者进行同样密切的随访。
