戈谢病胸部CT特征分析  

Analysis of CT features of chest in Gaucher disease

作　　者：李笛 陶晓娟[1] 张宁宁[1] 周作福[2] 申红卫[3] 张永红[4] 徐保平[5] 彭芸[1] Li Di;Tao Xiaojuan;Zhang Ningning;Zhou Zuofu;Shen Hongwei;Zhang Yonghong;Xu Baoping;Peng Yun(Department of Radiology,Beijing Children′s Hospital,Capital Medical University,Beijing 100045,China;Department of Radiology,Fujian Maternal and Child Health Hospital,Fuzhou 350001,China;Department of Radiology,Beijing Northen Hospital of Weaponry Industry,Beijing 100089,China;Department of Hematology,Beijing Children′s Hospital,Capital Medical University,Beijing 100045,China;Department of Respiration,Beijing Children′s Hospital,Capital Medical University,Beijing 100045,China)

机构地区：[1]国家儿童医学中心首都医科大学附属北京儿童医院放射科,100045 [2]福建省妇幼保健院放射科,福州350001 [3]兵器工业北京北方医院放射科,100089 [4]国家儿童医学中心首都医科大学附属北京儿童医院血液科,100045 [5]国家儿童医学中心首都医科大学附属北京儿童医院呼吸科,100045

出　　处：《中华放射学杂志》2020年第1期23-27,共5页Chinese Journal of Radiology

基　　金：北京市医院管理局儿科学科协同发展中心儿科专项创新推广项目(XTCX201814);北京市医院管理局儿科学科协同发展中心儿科专项子课题(XTZD20180104)。

摘　　要：目的探讨戈谢病患者胸部CT的影像表现,以提高对本病肺部浸润的诊断能力。方法回顾性搜集2003年5月至2018年10月在北京儿童医院诊治的戈谢病患者43例,其中男25例,女18例。所有患者均行常规胸部CT检查,分析和描述其肺部间质和实质等影像表现。结果43例戈谢病患者中,有20例胸部CT表现异常。肺部呈弥漫性小叶间隔增厚10例,主要分布在双肺下叶;肺部呈单叶段或多叶段的磨玻璃征象5例;细小结节影2例,表现为大小不等的类圆形结节影;肺纤维化者1例,以左肺上叶更为显著。其他表现包括肺囊泡3例、局限性胸膜增厚2例、气胸1例、肺动脉高压1例、胸腺增大12例。戈谢病患者出现肺部病变者以10~14岁居多,其中,小叶间隔增厚与胸腺增大等征象多见,在该年龄区间内分别各有5例。结论戈谢病患者半数累及肺部,肺部表现多样,多表现为弥漫间质病变,主要征象为小叶间隔增厚和磨玻璃样透光度减低,与戈谢细胞浸润病理一致,但不具备特异性,需要结合临床进行诊断,并注意与其他疾病引起的肺浸润相鉴别。Objective To explore the imaging manifestations of thoracic CT in patients with Gaucher disease(GD)in order to improve the diagnostic ability.Methods Forty-three patients with GD were collected from May 2003 to October 2018 in Beijing Children′s Hospital,including 25 males and 18 females,aged from 10 to 34 years,with an average age of(21±6)years.All the patients underwent routine chest CT examinations,and analysis and description of pulmonary interstitial and parenchyma imaging manifestations were performed.Results Among the 43 GD patients,20 patients presented with abnormal chest CT findings:10 showed diffuse interlobular septa thickening,mainly distributed in the lower lobes of both lungs;5 showed ground glass opacities in a single or multiple lobes of the lung.There were 2 cases with small nodules,which showed round-like nodules of different sizes.One case had pulmonary fibrosis,especially in the left upper lobe.Other manifestations included bullae in 3 cases,localized pleural thickening in 2 cases,pneumothorax in 1 case;pulmonary hypertension in 1 case and thymus enlargement in 12 cases.Most of the GD patients had pulmonary lesions between 10 and 14 years old.The signs of interlobular septa thickening and thymus enlargement were common,with 5 cases in each age group.Conclusions GD involves the lungs in half of the patients.The manifestations of the lungs are diverse,and most of them are diffuse interstitial lesions.The main signs are interlobular septal thickening and ground glass opacity,which are consistent with the pathology of Gaucher cell infiltration.But the signs are not specific,the diagnosis should be made in combination with the clinical information,and attention should be paid to the differentiation of lung infiltration caused by other diseases.

关 键 词：戈谢病 肺 体层摄影术 X线计算机 

分 类 号：R72[医药卫生—儿科] R816[医药卫生—临床医学]