儿童特发性肺含铁血黄素沉着症36例临床分析

目的 分析儿童特发性肺含铁血黄素沉着症(IPH)临床特点、治疗及预后,为临床防治该病提供依据。方法 回顾性分析2005年1月-2016年6月在西京医院首次诊断并完成1年以上随访的36例IPH患儿的临床资料,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 36例患儿中,男20例、女16例,发病中位年龄4.3岁,确诊中位年龄6岁,主要临床表现为咳嗽、咯血、气促、贫血、发热、乏力等。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＞O.05)。误诊时间(OR=0.084,95%CI:0.01～0.93)及初诊咯血史(OR=0.02,95%CI:0～0.461)是IPH反复发作的独立危险因素。结论 IPH临床表现多样,易误诊,糖皮质激素可较好的控制急性期病情,延长糖皮质激素治疗时间对IPH复发无影响。应尽早诊断,及时正规治疗,有助于缓解症状,延缓肺纤维化进程,改善预后。

Clinical analysis of idiopathic pulmonary hemosiderosis in children

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.