儿童特发性肺含铁血黄素沉着症的影像学诊断

Imaging Diagnosis 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)的影像表现。方法:回顾性分析10例经病理证实的特发性肺含铁血黄素沉着症的X线及CT表现。结果:初诊误诊为支气管肺炎3例,肺结核1例,1例先误诊为肺炎后又误诊为肺结核,误诊率50%。X线表现双肺片絮状阴影及磨玻璃样改变3例;双肺中下肺野斑片状影,肺纹理增多模糊3例;弥漫分布粟粒状、小结节状影伴磨玻璃样改变2例;弥漫分布网织状阴影2例。伴肺门影增大模糊3例、心影增大1例等。普通CT表现为两中下肺呈片絮状、小结节状阴影1例;弥漫分布粟粒状阴影伴磨玻璃样改变者2例。2例见空气支气管征,1例见多发小气囊。HRCT表现为两肺内弥漫分布网结节影1例,表现两肺网织影及散在小斑片状阴影、小叶间隔增厚1例。结论:对本病的认识不足是误诊的主要原因,X线及CT检查是发现并提示IPH的基本检查手段,影像表现结合临床,尤其是小儿痰、胃液中发现含铁血黄素巨噬细胞是提高确诊率的关键。

Objective.. To study the imaging findings of idiopathic pulmonary hemosiderosis （IPH） in pediatric patients. Methods：The manifestations of X-ray and CT of 10 pediatric cases with pathologically proved IPH were retrospectively reviewed. Results .. The preliminary misdiagnosis rate of IPH was 50 %. The misdiagnoses were as follows： bronchial pneumonia in 3 cases,pulmonary tuberculosis in 1 case and another one case initially misdiagnosed as pneumonia and later as pulmonary tuberculosis. X-ray signs presented as diffuse patchy consolidation and ground glass shadows （n= 3）, spotted shadows and patches in the bilateral middle and lower fields with blurred lung markings （n=3）,diffuse miliary and micronodular shadows with ground glass opacities （n= 2）, diffuse reticular shadows （ n= 2）, enlarged and blurred pulmonary hila （n=3） as well as enlarged heart shadow （n= 1）. In conventional CT, features of spotted and patchy as well as micronodular shadows in the middle and lower lobes of bilateral lungs （n=1） ;diffuse miliary and ground glass shadows （n= 2）, air bronchograms （n= 2）, and multiple pulmonary air-sacs （n = 1 ） were demonstrated. On HRCT images, diffused and reticular distribution of nodules （n： 1） and diffuse reticular shadows with patches and miliary nodules as well as thickened interlobular septa （n= 1） were noted. Conclusion：Clinically, the major cause of the preliminary misdiagnoses of IPH in pediatric patients was probably the unawareness of the disease. X-ray and CT examinations were the elementary modalities. Imaging features in combination with clinical information,particularly findings of hemosiderin within phagocytes in sputum and gastric juice,were keys to improve diagnostic accuracy of the disease.