Santamaria Francesca, Montella Silvia, Tiddens Harm A W M, Guidi Guido, Casotti Valeria, Maglione Marco, de Jong Pim A
Department of Pediatrics, Federico II University, Naples, Italy.
Department of Diagnostic Imaging, Radiology Service, AORN Antonio Cardarelli, Naples, Italy.
Chest. 2008 Aug;134(2):351-357. doi: 10.1378/chest.07-2812. Epub 2008 Apr 10.
High-resolution CT (HRCT) scan data on primary ciliary dyskinesia (PCD) related lung disease are scarce.
We evaluated the lung disease in children and adults with PCD by a modified Brody composite HRCT scan score to assess the prevalence of the structural abnormalities; to evaluate the correlation among HRCT scan scores, spirometry findings, and clinical data; and to compare the PCD scores with those of age-matched and sex-matched cystic fibrosis (CF) patients.
Twenty PCD patients (age range, 4.6 to 27.5 years) underwent HRCT scanning, spirometry, and deep throat or sputum culture. A modified Brody score was used to assess bronchiectasis, mucous plugging, peribronchial thickening, parenchyma abnormalities, and mosaic perfusion.
The total HRCT scan score was 6% of the maximal score (range, 0.5 to 25.5). Subscores were as follows: bronchiectasis, 5.6%; mucous plugging, 5.6%; peribronchial thickening, 8.3%; parenchyma, 3%; and mosaic perfusion, 0%. The prevalence of lung changes were as follows: bronchiectasis, 80%; peribronchial thickening, 80%; mucous plugging, 75%; parenchyma, 65%; and mosaic perfusion, 45%. Sixteen of 19 PCD patients had positive culture findings, and the most common pathogen found was Haemophilus influenzae (84%). The total HRCT scan score was significantly related to age (p = 0.006), FEV(1) (p = 0.02), and FVC (p = 0.02). The bronchiectasis subscore was significantly related to FEV(1) (p = 0.04) and FVC (p = 0.03). In CF patients, the total HRCT scan score was significantly higher than that in PCD patients (p = 0.02).
PCD patients show significantly lower pulmonary HRCT scan scores than CF patients. The PCD total and bronchiectasis scores correlate with spirometry findings. The PCD HRCT scan score might be used for longitudinal assessment and/or represent an outcome surrogate in future studies.
关于原发性纤毛运动障碍（PCD）相关肺部疾病的高分辨率CT（HRCT）扫描数据稀缺。
我们通过改良的布罗迪综合HRCT扫描评分评估PCD患儿和成人的肺部疾病，以评估结构异常的患病率；评估HRCT扫描评分、肺功能检查结果和临床数据之间的相关性；并将PCD评分与年龄和性别匹配的囊性纤维化（CF）患者的评分进行比较。
20例PCD患者（年龄范围4.6至27.5岁）接受了HRCT扫描、肺功能检查以及深部咽喉或痰液培养。采用改良的布罗迪评分评估支气管扩张、黏液嵌塞、支气管周围增厚、实质异常和马赛克灌注。
HRCT扫描总评分占最大评分的6%（范围0.5至25.5）。各分项评分如下：支气管扩张5.6%；黏液嵌塞5.6%；支气管周围增厚8.3%；实质3%；马赛克灌注0%。肺部改变的患病率如下：支气管扩张80%；支气管周围增厚80%；黏液嵌塞75%；实质65%；马赛克灌注45%。19例PCD患者中有16例培养结果呈阳性，最常见的病原体是流感嗜血杆菌（84%）。HRCT扫描总评分与年龄（p = 0.006）、第1秒用力呼气容积（FEV₁）（p = 0.02）和用力肺活量（FVC）（p = 0.02）显著相关。支气管扩张分项评分与FEV₁（p = 0.04）和FVC（p = 0.03）显著相关。在CF患者中，HRCT扫描总评分显著高于PCD患者（p = 0.02）。
PCD患者的肺部HRCT扫描评分显著低于CF患者。PCD的总分和支气管扩张评分与肺功能检查结果相关。PCD的HRCT扫描评分可用于纵向评估和/或代表未来研究中的替代结局指标。
