Department of Pediatrics, Federico II University, Via Pansini 5, Naples, Italy.
Pediatr Pulmonol. 2012 May;47(5):498-504. doi: 10.1002/ppul.21569. Epub 2011 Oct 17.
Despite its extensive use, there is no evidence that spirometry is useful in the assessment of progression of lung disease in primary ciliary dyskinesia (PCD). We hypothesize that high-resolution computed tomography (HRCT) is a better indicator of PCD lung disease progression than spirometry. We retrospectively evaluated two paired spirometry and HRCT examinations from 20 PCD patients (age, 11.6 years; range, 6.5-27.5 years). The evaluations were performed in stable state and during unstable lung disease. HRCT scans were scored blind by two raters. Compared to the first assessment, at the second evaluation spirometry did not change while HRCT scores significantly worsened (P < 0.01). Age was significantly related to HRCT total (r = 0.5; P = 0.02) and bronchiectasis scores (r = 0.5; P = 0.02). At both evaluations, HRCT total score correlated with FEV(1) (r = -0.5, P = 0.01; r = -0.7, P = 0.001, respectively) and FVC Z scores (r = -0.6, P = 0.006; r = -0.7, P = 0.001, respectively), and bronchiectasis score was related to FEV(1) (r = -0.5, P = 0.03; r = -0.6; P = 0.002, respectively) and FVC Z scores (r = -0.6, P = 0.008; r = -0.7, P = 0.001, respectively). No relationship was found between the change in HRCT scores and the change in spirometry. In PCD, structural lung disease may worsen despite spirometry being stable.
尽管广泛应用，但没有证据表明肺量测定在原发性纤毛运动障碍（PCD）的肺部疾病进展评估中有用。我们假设高分辨率计算机断层扫描（HRCT）是比肺量测定更好的PCD 肺部疾病进展指标。我们回顾性评估了 20 名 PCD 患者的两次配对肺量测定和 HRCT 检查（年龄，11.6 岁；范围，6.5-27.5 岁）。评估在稳定状态和不稳定的肺部疾病期间进行。HRCT 扫描由两名评分者进行盲法评分。与第一次评估相比，在第二次评估时，肺量测定没有变化，而 HRCT 评分明显恶化（P<0.01）。年龄与 HRCT 总分（r=0.5；P=0.02）和支气管扩张评分（r=0.5；P=0.02）显著相关。在两次评估中，HRCT 总分均与 FEV1（r=-0.5，P=0.01；r=-0.7，P=0.001）和 FVC Z 评分（r=-0.6，P=0.006；r=-0.7，P=0.001）呈负相关，支气管扩张评分与 FEV1（r=-0.5，P=0.03；r=-0.6；P=0.002）和 FVC Z 评分（r=-0.6，P=0.008；r=-0.7，P=0.001）呈负相关。HRCT 评分的变化与肺量测定的变化之间没有关系。在 PCD 中，尽管肺量测定稳定，但结构性肺部疾病可能会恶化。
