婴幼儿尼曼-皮克病肺部病变高分辨率CT表现

目的 观察婴幼儿尼曼-皮克病肺部病变的高分辨率CT（HRCT）表现。方法 回顾性分析11例经基因检测/骨髓穿刺活检/酶学活性检测确诊尼曼-皮克病患儿的胸部HRCT表现,包括气道病变征象、间质病变征象、肺泡病变征象及其他肺或肺外改变。对双肺主动脉弓层面、气管隆嵴层面及膈面上层面的HRCT异常及累及范围分别进行评分。比较不同种类病变及累及范围的差异。结果 气道病变和间质病变发生率均为100%（11/11）,肺泡受累为81.82%（9/11）;上述3类病变累及范围差异有统计学意义（H=10.57,P<0.01）,而左、右肺野病变累及范围相近（U=27.5,P=0.64）。气道病变所致各种异常累及范围差异有统计学意义（H=8.81,P=0.03）,以支气管壁增厚和树芽征范围最广。肺间质病变所致小叶间隔增厚、小叶内线及小叶中心阴影累及范围差异无统计学意义（H=5.67,P=0.06）。9例患儿肺部见磨玻璃影,累及范围相近。结论 尼曼-皮克病肺部病变HRCT表现以肺间质和肺泡受累为主,间质病变多见于上肺野和下肺野,气道病变多见于上肺野,而肺泡病变分布较广泛、均匀。

High resolution CT manifestations of pulmonary lesions in infants with Niemann-Pick disease

Objective To observe the high resolution CT (HRCT) manifestations of pulmonary lesions in infants with Niemann-Pick disease. Methods HRCT manifestations of 11 infants with Niemann-Pick disease confirmed by gene test/bone marrow biopsy/enzymatic activity test were retrospectively analyzed, and the airway lesions, interstitial lesions, alveolar lesions and other pulmonary or extrapulmonary changes were observed. The involvement range of HRCT abnormalities in bilateral pulmonary aortic arch plane, tracheal carina plane and diaphragmatic plane were scored, respectively. The ranges of involvements of different kinds of lesions and different HRCT signs abnormalities were compared. Results Airway and interstitial lesions were found in all 11 cases (100%, 11/11), and alveolar lesions were detected in 9 cases (81.82%, 9/11). There were statistical differences of involvement ranges among airway lesions, interstitial lesions and alveolar lesions (H=10.57, P<0.01). The involvement ranges of pulmonary lesions ion left and right lobe lung field were similar (U=27.5, P=0.64). There were significant differences of the involvement ranges of of various signs abnormalities caused by airway lesions (H=8.81, P=0.03), and bronchial wall thickening and "tree buds" most distributed widely involved. Meanwhile, there was no significant difference of the involvement ranges of interlobular septal thickening, intralobular line and centrilobular shadow caused by interstitial lesions (H=5.67, P=0.06). Ground-glass opacity (GGO) was observed in 9 cases, and the involvements in each side lung field were similar. Conclusion HRCT of pulmonary lesions of Niemann-Pick disease in infants mainly manifested as interstitial and alveolar lesions, and the interstitial lesions mainly presented in the upper and lower lung fields, airway involvements mainly presented in the upper lung field, alveolar lesions distributed widely and uniformly.