Résumé
Objective To describe the clinical characteristics of idiopathic pulmonary hemosiderosis (IPH),and to investigate the possible risk factors for poor prognosis.Methods The clinical data of 41 patients with IPH were retrospectively analyzed,22 cases were divided into survival group and death group according to the follow-up data and prognosis,and the related clinical factors in 2 groups were statistically analyzed.Results Of the 41 patients with IPH,14 cases were male,27 cases female.The median age of onset was 3.8 years,and the diagnosed median age was 4.6 years.The common clinical features of the 41 children with IPH included pale,cough,hemoptysis,fever,and fatigue,in 34 cases (82.9％),27 cases (65.8％),16 cases (39.0％),13 cases (31.7％) and 11 cases (26.8％) children,respectively.Initial symptoms included pale,cough,hemoptysis,in 27 cases (65.9％),13 cases (31.7 ％),and 11 cases (26.8％) children,respectively.Accessory examinations revealed microcytic hypochromic anemia (average hemoglobin concentration was 65.2 g/L),and 40 patients (97.6％)had an abnormal chest X-ray.Forty patients were treated with glucocorticoids,and all of them had symptom remission.Twenty-two patients were followed up for 6 months to 9 years,and 6 cases (27.3％)died during follow-up and 16 cases (72.7％) still alive.There were no statistically significant differences between death group and survival group as to age of onset,gender distribution,age on diagnosis,degree of anemia,and clinical features(all P ＞ 0.05).The incidence of jaundice was significantly different between the 2 groups (P ＜ 0.05).Conclusions IPH has diverse clinical manifestations and a high rate of misdiagnosis.A routine chest Xray film should be taken for patients with moderate to severe microcytic hypochromic anemia.The conditions of some pediatric patients can be stabilized with glucocorticoids treatment,but the associated mortality rate remains high.History of jaundice may be a risk factor for poor prognosis.