关键词: 特发性肺含铁血黄素沉着症, 临床分析, 儿童

Abstract: Objective To analyze the clinical characteristics,treatment,and prognosis of idiopathic pulmonary hemosiderosis(IPH),and to provide evidence for clinical prevention and treatment of the disease. Methods Totally 36 IPH cases who were hospitalized from January 2005 to June 2016 were collected and followed up for more than 1 year.Their clinical data were retrospectively analyzed.According to the duration of oral prednisone treatment,the patients were divided into different groups and the relapse ratios were compared among groups.Based on the existence of relapse,the patients were divided into two groups to analyze the risk factors of relapse. Results In 36 cases(20 males,16 females),the median age of the onset was 4.3 years old and the median age of diagnosis was 6 years old.The main clinical manifestations were anemia,cough,hemoptysis,shortness of breath,fever and fatigue.Glucocorticoid could control the acute symptoms of IPH.Among the patients with oral prednisone treatment≤ 1 year,~ 2 years,~3 years and ＞3 years groups,there were no significant diferences of relapse ratio (P>0.05).The misdiagnosis time (OR=0.08,95%CI:0.01～0.93)and hemoptysis at first visit (OR=0.02,95%CI:0～0.461) might be the independent risk factors of relapse. Conclusions IPH has diverse clinical manifestatios　and easy to be misdiagnosed.Glucocorticoid is effective to control the acute symptoms of IPH.Prolonging the course of glucocorticoid therapy can not reduce the relapse of IPH.To diagnose and start treat in time would be good to relieve symptoms,delay the process of pulmonary fibrosis and improve prognosis.

Key words: idiopathic pulmonary hemosiderosis, clinical characteristics, children