Mendelson David S, Wasserstein Melissa P, Desnick Robert J, Glass Ronald, Simpson William, Skloot Gwen, Vanier Marie, Bembi Bruno, Giugliani Roberto, Mengel Eugen, Cox Gerald F, McGovern Margaret M
Dept of Radiology, Mount Sinai School of Medicine, One Gustave L. Levy Place, New York, NY 10029, USA.
Radiology. 2006 Jan;238(1):339-45. doi: 10.1148/radiol.2381041696. Epub 2005 Nov 22.
To evaluate findings at radiography, computed tomography (CT), and pulmonary function testing in patients with type B Niemann-Pick disease.
The study was approved by the institutional review board or ethics committee at each study site and was compliant with HIPAA at the U.S. site. Written informed consent was obtained from each patient or guardian and minor assent was obtained from all children before any study-related procedures. Pulmonary involvement in 53 patients (27 male and 26 female patients; age range, 7-65 years; mean age, 23.3 years) with type B Niemann-Pick disease was evaluated with imaging and pulmonary function tests. All patients underwent chest radiography and thin-section CT, and images were independently interpreted by one of two radiologists. Spirometry (forced vital capacity [FVC] and forced expiratory volume in 1 second [FEV1]) was performed and diffusing capacity of lung for carbon monoxide (Dlco) was evaluated in all patients who could comply. A score for the degree of interstitial lung disease was derived at both radiography and CT, and the CT scores were then compared with results of pulmonary function testing and patient age by means of linear regression. CT scores were compared between the upper and lower lung zones by using the Wilcoxon signed rank test.
Chest radiography and CT, respectively, revealed interstitial lung disease in 47 (90%) and 51 (98%) of the 52 patients who completed both imaging examinations. There was a basilar predominance of interstitial lung disease at CT. Six patients had pulmonary nodules, one of which was calcified at chest radiography. There were no statistically significant correlations between interstitial lung disease score at CT and age or percentage predicted FVC, FEV1, or Dlco values.
Although pulmonary function test indexes may be abnormal, imaging findings do not necessarily correlate with pulmonary function in patients with type B Niemann-Pick disease.
评估B型尼曼-匹克病患者的X线摄影、计算机断层扫描（CT）及肺功能检查结果。
本研究经各研究地点的机构审查委员会或伦理委员会批准，在美国的研究地点符合《健康保险流通与责任法案》（HIPAA）要求。在进行任何与研究相关的程序之前，均获得了每位患者或监护人的书面知情同意书，并获得了所有儿童的未成年同意。对53例（27例男性和26例女性患者；年龄范围7 - 65岁；平均年龄23.3岁）B型尼曼-匹克病患者的肺部受累情况进行了影像学和肺功能检查评估。所有患者均接受了胸部X线摄影和薄层CT检查，图像由两名放射科医生之一独立解读。对所有能够配合的患者进行了肺活量测定（用力肺活量[FVC]和1秒用力呼气量[FEV1]），并评估了肺一氧化碳弥散量（Dlco）。在X线摄影和CT检查时均得出了间质性肺疾病程度的评分，然后通过线性回归将CT评分与肺功能检查结果及患者年龄进行比较。采用Wilcoxon符号秩检验比较上下肺区的CT评分。
在完成两项影像学检查的52例患者中，胸部X线摄影和CT分别显示47例（90%）和51例（98%）存在间质性肺疾病。CT显示间质性肺疾病以肺底部为主。6例患者有肺结节，其中1例在胸部X线摄影时呈钙化。CT上的间质性肺疾病评分与年龄或预测的FVC、FEV1或Dlco值的百分比之间无统计学显著相关性。
虽然肺功能检查指标可能异常，但B型尼曼-匹克病患者的影像学表现不一定与肺功能相关。
