摘要目的 总结儿童特发性肺含铁血黄素沉着症(idiopathic pulmonary bemosiderosis,IPH)的临床表现、实验室检查、影像学、病理学特点,并探讨诊断、治疗方法和预后.方法 本例患者进行实验室、影像学、深部吸痰并行组织病理学检查确诊,糖皮质激素治疗并随访,探讨本例患者并结合国内外相关报道文献进行复习.结果 临床表现主要是反复发作咯血、咳嗽、贫血,部分心慌、胸闷、乏力,肺部体征不明显,影像学改变可见肺部弥漫性毛玻璃样改变,相关免疫学检查(如抗核抗体、抗中性粒细胞包浆抗体)均阴性,血分析呈小细胞低色素性贫血,血清铁降低,胆红素升高,反复深部痰液可找到含铁血黄素巨噬细胞.结论 儿童特发性肺含铁血黄素沉着症临床表现多样,需与缺铁性贫血、支气管肺炎、肺结核相鉴别,容易误诊、漏诊,痰、胃液及支气管肺泡灌洗液活检可提高阳性率,应加强对本病的认知,及早诊断和干预;长时间糖皮质激素治疗后可使病情处于较稳定状态;预后不良.

abstractsObjective To summarize the clinical manifestation, laboratory examination, radiological and pathological characteristics of idiopathic pulmonary hemosiderosis in children and discusses the diagnosis,treatment and prognosis. Methods The patient for laboratory, imaging, deep sucking phlegm parallel histopathological examination confirmed, corticosteroid and followed-up, discusse the patient and combined domestic and foreign literature review reports. Results Clinical manifestations of main were recurrent haemoptysis,cough, anemia, partial bosom frowsty, flustered, feeble, no obvious signs in lungs. Radiculopathy lung diffuse ground-glass appearance was changed, relevant immunological tests (e. g, antinuclear antibodies, neutrophils patina antibodies) were negative, blood analysis was a hypochromic microcytic anemia, elevated bilirubin, serum iron reduces, deep sputum containing blood can be found macrophages. Conclusion Children idiopathic pulmonary hemosiderin clinical manifestations of diversity, with iron deficiency anemia, bronchopneumonia, tuberculosis, it is easy to be misdiagnosed, distinguish repeatedly, the diagnosis of gastric juice and bronchoaleolar laage biopsy can improve the rate of disease, should strengthen the cognition, early diagnosis and intervention. Long-range cocorticoid therapy can make an illness in a stable state. The poor prognosis.