儿童特发性肺含铁血黄素沉着症临床影像分析

目的:探讨特发性肺含铁血黄素沉着症(IPH)的胸部影像特点.方法:回顾性分析本院1999-2005年临床确诊的18例IPH影像资料.结果:胸部X线表现:①肺纹理增强15例;②肺野内片絮状阴影或磨玻璃样影12例;③双肺弥散分布的粟粒状、小结节影6例;④双肺弥漫境界不清的网织状影8例,其中2例伴心脏增大,1例伴肺动脉段隆鼓.高分辨率CT(HRCT)显示:双肺弥漫性密度增高,呈片絮状及磨玻璃样改变2例,其中1例实变影内见支气管充气征;双肺弥漫分布粟粒状、小结节状影1例;双肺小叶间隔增厚2例,其中弥漫增厚1例;轻度蜂窝状肺改变1例;心影增大1例.结论:胸片是发现IPH的重要方法,HRCT对病灶的显示更为清晰,只要注意将影像表现与临床表现、实验室检查结合分析,尤其在痰、胃液或肺组织中找到含铁血黄素巨噬细胞,可明确诊断.

Clinical and Radiological Analysis of Idiopathic Pulmonary Hemosiderosis in Children

Objective:To study the imaging characteristics and pathologic findings of idiopathic pulmonary hemosiderosis(IPH).Methods: The clinical data and imaging findings of 18 cases with IPH treated in our hospital between 1999 and 2005 were retrospectively analyzed.Results: On chest radiography,enhanced lung markings(n=15),flake consolidation and ground-glass shadows(n=12),diffuse miliary and micronodular shadows(n=6),and diffuse reticular shadows(n=8) were shown in both lungs,and enlarged heart shadow in 2 cases and bulged the segment of pulmonary artery in 1 case were seen.On HRCT,diffuse flake consolidation and ground-glass shadows(n=2) and air bronchogram(n=1),diffuse miliary and micronodular shadows(n=1),and thickened interlobular septum(n=2) were demonstrated.Conclusion: Chest film is the important method to detect IPH,while HRCT may provide more information on foci of interest.Combined analysis of imaging findings,clinical manifestation,and laboratory results,particularly the presence of hemosiderin macrophagocyte in sputum,gastric juice or lung tissue may provide a confirmed diagnosis of IPH.