目的 分析儿童特发性肺含铁血黄素沉着症（idiopathic pulmonary hemosiderosis,IPH）的临床特点、治疗及预后。方法 对2010年1月—2021年6月海南省2家三甲医院收治的12例确诊IPH患儿的临床资料进行回顾性分析。结果 12例患儿,入院前有抗感染治疗效果欠佳病史的患儿有11例。其中男性患儿8例,女性患儿4例。年龄<3岁者3例,≥3岁~10岁者8例,>10岁者1例。临床表现为咳嗽、咳痰的患儿有8例,面色苍白的患儿有6例,咯血的患儿有7例,呕血的患儿有2例,胸闷的患儿有1例。12例患儿行含铁血黄素试验结果均阳性,其中痰液含铁血黄素试验阳性9例,胃液含铁血黄素试验阳性1例,尿液含铁血黄素试验阳性1例,肺泡灌洗液含铁血黄素试验阳性1例。12例患儿均行胸部影像学检查,结果回报均检测出肺部病变。结论 有反复咳嗽、咯血、面色苍白病史且经抗感染、纠正贫血治疗效果欠佳的患儿,除外其他继发性肺出血性疾病后,需考虑IPH。完善含铁血黄素沉着试验、胸部影像学检查可帮助诊断,激素治疗是治疗本病的有效措施。

Objective To analyze the clinical features, treatment and prognosis of children with idiopathic pulmonary hemosiderosis (IPH).Methods From January 2010 to June 2021, the clinical data of 12 children with confirmed IPH who were admitted to two tertiary hospitals in Hainan Province were retrospectively analyzed.Results Among the 12 cases, 11 cases had a history of poor anti-infective treatment before admission. Among the 12 cases , 8 cases were male and 4 cases were female. There were 3 patients aged<3 years old, 8 patients aged≥3 to 10 years old, and 1 patient aged>10 years. For clinical manifestations, there were 8 cases with cough and sputum, 6 cases with pale complexion, 7 cases with hemoptysis, 2 cases with hematemesis, and 1 case with chest tightness. The hemosiderin test results of 12 children were all positive, among which 9 cases were positive in sputum hemosiderin test, 1 case was positive in gastric juice hemosiderin test, 1 case was positive in urine hemosiderin test, and 1 case was positive in alveolar lavage test. Liquid hemosiderin test was positive in 1 case. All 12 children underwent chest imaging examination, and the results showed that pulmonary lesions were all detected.Conclusions Children with a history of repeated cough, hemoptysis, and pale complexion and poor treatment effect after anti infection and correction of anemia, and after ruling out other secondary pulmonary hemorrhagic disease, should consider being affected by IPH. The improvement of hemosiderin test and chest imaging examination can help the diagnosis, and hormone therapy is an effective measure to treat this disease.