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特发性肺含铁血黄素沉着症是一种少见、病因不明、好发于儿童的以弥散性肺泡毛细血管反复出血、肺间质含铁血黄素沉着为显著特点的疾病。反复咯血、缺铁性贫血和弥散性肺浸润三联征是其特征性表现。经铁染色检测痰、胃液、支气管肺泡灌洗液或肺组织含铁血黄素巨噬细胞为重要的诊断方法,高分辨率CT对痰液检查正常的患者具有一定的诊断意义,有条件者应行肺组织活检,除外其他肺出血性疾病。免疫抑制治疗为主要治疗方法,对于肺出血威胁生命的患者采取积极的干预治疗,可降低病死率。
Idiopathic pulmonary hemosiderosis is a rare disease of unknown etiology that occurs frequently in children with disseminated alveolar capillaries that recurrent bleeding and interstitial lung hemosiderosis. Repeated hemoptysis, iron deficiency anemia and diffuse pulmonary triad is the characteristic manifestation. Detection of sputum by iron staining, gastric juice, bronchoalveolar lavage fluid or lung tissue hemosiderin macrophage is an important diagnostic method, high-resolution CT sputum examination of patients with normal diagnostic significance, the condition should be Lung biopsy, except for other pulmonary hemorrhagic diseases. Immunosuppressive therapy as the main treatment, for life-threatening pulmonary hemorrhage in patients with active intervention to reduce mortality.