摘要
特发性肺含铁血黄素沉着症(idiopathic pulmonary hemosiderosis,IPH)是一种罕见的,以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。其病因及发病机制尚未完全明了,过敏反应、霉菌感染等可能诱发IPH,免疫因素在IPH发病中起着至关重要的作用,最新的研究已经发现细胞因子和自身免疫性血管炎在IPH发病中有一定作用。其临床表现多样,无明显特异性,主要表现为反复发作的咯血、气促和贫血。痰涂片或肺泡灌洗液经铁染色为重要的诊断方法,高分辨CT对于痰液检查正常的患者具有一定的诊断意义,免疫抑制治疗为主要治疗方法,肺移植的可行性存在争议。我们较为详细地总结了近年来有关于本病的病因、发病机制、临床特点、诊断治疗及预后的研究进展。
Idiopathic pulmonary hemosiderosis （IPH） is a rare disease characterized by recurrent episodes of diffuse alveolar hemorrhage and deposition of hemosiderin in alveolar interstice. Causes and pathogenesis of IPH are still unclear. Allergic response and fungi infection might be involved, and immunologic factors are considered as the most important pathogenesis. Recent studies have found that the cytokines and autoimmune vasculitis play an important role in inducing IPH. The clinical manifestations varied without specificity. The main clinical manifestations are recurrent episodes of hemoptysis, dyspnea and anemia. Sputum examination under microscope or bronchoalveolar lavage fluid test by iron staining is an important diagnostic method. High resolution computerized tomography has diagnostic value for the patients with normal sputum examination. Immunosuppressive therapy is the main treatment. The feasibility of lung transplantation is still controversy. Recent advances on the pathogenesis, clinical features, diagnosis, treatment and prognosis of IPH were reviewed in detail by author.
出处
《临床儿科杂志》
CAS
CSCD
北大核心
2007年第3期232-235,共4页
Journal of Clinical Pediatrics
