Analysis Of 12 Death Case Of Idiopathic Pulmonary Hemosiderinosis

Objective: Analyze the clinical characteristics and causes of death of children with idiopathic pulmonary hemosiderosis(IPH),improve and emphasize the importance of early diagnosis and standardized treatment of IPH,and reduce the mortality of the disease.Methods: The clinical manifestations,diagnosis,treatment,and causes of death of 12 patients with IPH diagnosed and died in the hospital from 1993 to 2018 in the Department of Hematology and Oncology of Children’s Hospital Affiliated to Chongqing Medical University were retrospectively analyzed.Results: Among the 12 dead children,5 were male and 7 were female.The median age of onset was 1 years and 4 months,and the median duration of disease before diagnosis was 4.5 months.12 cases had anemia at the time of initial diagnosis,10 patients had severe / extreme anemia,7 patients had anemia with cough,and only 1 case had triple sign.Chest imaging at the first diagnosis showed 5 cases of ground glass like shadows,2 cases of patchy and stripe ghost images,3 cases of increased lung texture / light transmittance decreased slightly,1 case of acute massive exudative pulmonary hemorrhage and 1 case of extensivesubacute miliary lesions.11 children were treated with glucocorticoid immediately after diagnosis,including 6 cases of methylprednisolone pulse treatment,4 cases of standard dose,1 case of dexamethasone treatment.After 1-2 weeks of initial hormone therapy,8 patients achieved significant / partial remission,3 cases without remission.1 case delayed hormone treatment.5 patients died immediately after being hospitalized for the first time;7 relapsed repeatedly,4 died of acute pulmonary hemorrhage suddenly after infection during standard hormone therapy,and 2 patients had poor medical compliance(self-reducing hormone,oral Chinese medicine).The median duration of the course was 28 months,and the median survival time was 5 months after diagnosis.Conclusion: The clinical manifestations and chest imaging of children with IPH death are relatively serious.The main causes of death are misdiagnosis or failure to get timely diagnosis and treatment,insensitive or dependence to hormone,infection induce or aggravate pulmonary hemorrhage,and poor compliance.Therefore,health education of IPH should be strengthened,the rate of early diagnosis and treatment should be improved.Combining with other immunosuppressant treatment if necessary,controlling and avoiding respiratory tract infection,tapering hormones timely,and follow-up standardized according to doctor’s advice.