儿童特发性肺含铁血黄素沉着症影像学分析

Radiological analysis of idiopathic pulmonary hemosiderosis in children

目的探讨儿童特发性肺含铁血黄素沉着症（IPH）的胸部影像学特点。方法对我院2000年至2010年收治并临床确诊的16例特发性肺含铁血黄素沉着症患者的影像学及临床资料进行回顾性分析。结果胸部X线表现：16例肺野内弥漫分布有片絮状阴影与磨玻璃样改变，8例表现为双肺弥散分布的粟粒状、小结节影；5例有弥漫分布的网织状影。7例行CT扫描。高分辨率CT（HRCT）：7例两肺呈弥漫性分布片絮状实变影及磨玻璃样影，5例两肺弥漫分布粟粒状、小结节状影，4例可见多发小囊状影及网状影，2例可见支气管充气征，3例有小叶间隔增厚。结论X线平片是发现并提示IPH最基本的检查手段，影像学检查特别是HRCT与临床表现、实验室检查相结合分析，尤其是在痰或胃液中及肺泡灌洗液内查到含铁血黄素巨噬细胞，可以明确诊断。

Objective To study the imaging characteristics and pathologic findings of idiopathic pulmonary hemosiderosis （IPH）. Methods The clinical datas and imaging findings of 23 cases of IPH treated in our hospital between 2000 and 2010 were retrospectively analyzed. Results On plain film, flake consolidation and ground -glass shadows（ n = 16） ,diffuse miliary and micronodular shadows（ n = 8 ） , and difuse reticular shadows（ n = 5 ）were demonstrated in both lungs. On HRCT, diffuse flake consolidation and groundglass shadows （ n =7 ）, diffuse miliary and mieronodular shadows （ n = 5 ）and multiple follicular and reticular shadows （ n = 4）were displayed in both lungs. Air bronchogram was seen in 2 and thickened interlobular septum in 3 cases. Conclusions Chest film is the important method to detect IPH. Combined analysis of imaging findings（ HRCT）, clinical manifestation, and laboratory results, particularly the presence of hemosider in macrophagocyte in sputum, gastric juice or lung tissue may provide a confirmed diagnosis of IPH.