目的　分析特发性含铁血黄素沉着症（IPH）的临床特点、治疗及预后。方法　回顾性分析2006 年1 月至2014 年1 月首次诊断并完成6 个月以上随访的43 例IPH 患儿临床资料，根据单用泼尼松口服治疗时间进行分组，比较各组复发情况；根据病程中是否有反复发作进行分组，分析反复发作的危险因素。结果　43 例患儿中，男24 例、女19 例，确诊中位年龄4.1 岁（1.3~13.5 岁），主要临床表现为面色苍白、咳嗽、咯血、发热、乏力。急性发作期糖皮质激素治疗能控制症状，单用泼尼松口服治疗≤1年组、~2 年组、~3 年组及>3 年组中，不同时间的复发率差异无统计学意义（P>0.05）。病程长（OR=1.13，95% CI：1.03~1.24）及初诊咯血史（OR=9.91，95% CI：1.26~78.11）可能是IPH 反复发作的独立危险因素。肺活量（VC）、用力肺活量（FVC）、一秒用力呼气容积（FEV1）与IPH 病程呈负相关（r=–0.568~–0.659，P 均<0.01）。结论　IPH 临床表现多样，急性期糖皮质激素治疗有效，延长糖皮质激素治疗时间对IPH 复发无影响，IPH 反复发作可致限制性肺功能损害。

Objective　To analyze the clinical characteristics, treatment, and prognosis of idiopathic pulmonary hemosiderosis (IPH). Methods　The study included 43 IPH cases who were hospitalized from January 2006 to January 2014 and followed up for more than 6 months. 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 43 cases (24 males, 19 females) the median age of diagnosis was 4.1 years old (1.3-13.5 years). The main clinical manifestations were anemia, cough, hemoptysis, fever, and fatigue. Glucocorticoid can control the acute symptoms of IPH. Among the patients with oral prednisone treatment ≤ 1 year, -2 years, -3 years, and > 3 years group, there were no significant difference of relapse ratio (P>0.05). Long disease course (OR=1.13, 95%CI: 1.03-1.24) and hemoptysis at first visit (OR=9.91, 95%CI: 1.26-78.11) may be the independent risk factors of relapse. The vital capacity (VC), forced vital capacity (FVC), forced expiratory volume in 1s (FEV 1) were negatively correlated with the course of IPH (r=-0.568, -0.597, -0.659 respectively, P<0.001). Conclusions　The manifestations of IPH are varied. Glucocorticoid is effective to control the acute symptoms of IPH. Prolonging the course of glucocorticoid therapy cannot reduce the relapse of IPH. Repeated relapse of IPH can cause restrictive ventilation dysfunction.