Koumbourlis Anastassios C, Lee Donna J, Lee Ada
Division of Pediatric Pulmonology, Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University Medical Center, New York, NY, USA.
Pediatr Pulmonol. 2007 Jun;42(6):483-8. doi: 10.1002/ppul.20601.
We studied the changes in the patterns of lung function and somatic growth over time in children and adolescents (10.6 +/- 3.5 years at first test) with hemoglobin SS (Hb-SS) sickle cell disease (SCD).
Lung function and somatic growth were measured twice with an interval of 42.3 +/- 23.3 months in 45 children (25 females and 20 males) with Hb-SS SCD.
The lung volumes slightly decreased but remained borderline normal in both tests. All spirometric indices were within the normal range but significantly decreased (P<0.001) at the time of the second test indicating development of lower airway obstruction (forced expiratory volume in the first second (FEV(1)): 87 +/- 21 vs. 80 +/- 15; FEV(1)/forced vital capacity (FVC): 89 +/- 7 vs. 85 +/- 6; FEF(25-75): 89 +/- 32 vs. 76 +/- 24). "Normal" pattern of lung function was initially found in 56% of the patients, but in only 29% in the second test. In contrast, those with "obstructive" pattern increased from 22 to 44%, and those with "restrictive" pattern from 22 to 27%. There was no association between history of asthma and pattern of lung function. "Normal" Body Mass Index (BMI) was found in 64% of the patients, whereas 13% had "High" BMI and 22% "Low" BMI. The two latter patterns were associated with abnormal lung function but only patients with normal BMI showed actual decline overtime.
SCD is characterized by a predominantly obstructive pattern of lung function that increases in prevalence over time. There was no apparent causal relationship between the pattern of somatic growth and the pattern of lung function.
我们研究了血红蛋白SS（Hb-SS）镰状细胞病（SCD）患儿及青少年（首次检测时年龄为10.6±3.5岁）肺功能和身体生长模式随时间的变化。
对45例Hb-SS SCD患儿（25例女性，20例男性）进行了两次肺功能和身体生长测量，间隔时间为42.3±23.3个月。
两次检测中肺容量均略有下降，但仍处于临界正常范围。所有肺量计指标均在正常范围内，但在第二次检测时显著下降（P<0.001），表明下气道阻塞加重（第1秒用力呼气量（FEV(1)）：87±21 vs. 80±15；FEV(1)/用力肺活量（FVC）：89±7 vs. 85±6；呼气中期流速（FEF(25 - 75)）：89±32 vs. 76±24）。初始时56%的患者肺功能呈“正常”模式，但第二次检测时仅为29%。相比之下，“阻塞性”模式的患者从22%增至44%，“限制性”模式的患者从22%增至27%。哮喘病史与肺功能模式之间无关联。64%的患者体重指数（BMI）“正常”，13%的患者BMI“高”，22%的患者BMI“低”。后两种模式与异常肺功能相关，但只有BMI正常的患者肺功能随时间出现实际下降。
SCD的特征主要是肺功能呈阻塞性模式，且患病率随时间增加。身体生长模式与肺功能模式之间无明显因果关系。
