目的:了解特发性肺含铁血黄素沉着症(IPH)的临床特点,并探讨诊治和预后,提高该病的诊断水平。方法:分析我院2000年—2009年收治35例IPH患儿的临床表现、相关实验室检查及治疗方法。结果:35例患儿发病发病年龄10个月～15岁,均采用肾上腺皮质激素治疗,其中对16例急性出血期患儿采用甲基强的松龙联合应用大剂量丙种球蛋白治疗,肺部出血症状得到迅速控制,取得较好效果。结论:儿童IPH易误诊,早期诊断,长期正规治疗,定期随诊对尽早控制急性发作、减少反复次数、改善预后具有重要作用。 Objective: To understand the clinical features of idiopathic pulmonary hemosiderosis (IPH) and to discuss the diagnosis, treatment and prognosis and to improve the diagnosis of this disease. Methods: To analyze the clinical manifestations, laboratory tests and treatment of 35 children with IPH from 2000 to 2009 in our hospital. Results: The incidence of onset of 35 cases of children aged 10 months to 15 years were treated with adrenal cortex hormones, of which 16 cases of acute bleeding in children with methylprednisolone combined with high-dose gamma globulin treatment of lung Bleeding symptoms have been rapidly controlled and achieved good results. Conclusions: Misdiagnosis, early diagnosis, long-term regular treatment and regular follow-up of children with IPH are very important for early control of acute exacerbation, reduction of repetition frequency and improvement of prognosis.