The Clinical Study Of Pediatric Idiopathic Pulmonary Hemosiderosis

Objective:To summarize clinical characteristics of idiopathic pulmonary hemosiderosis(IPH)and analyze the etiopathogenisis,diagnosis,experiences in therapy and risk factors of IPH.Methods:84 cases with IPH from January 1980 to January 2007 were retrospectively analysized by patients' history,clinical symptoms,results of laboratory tests,diagnosis,therapy and other related indexes.A follow-up was taken in 34 patients.The death time and the causes of the deaths and the patient's conditions of the survivals were collected.Take investigations of the survivals. Compared the information of the deaths and the survivals.Results:The age of onset was(4.6±3.3)year.The results of immune system tests showed disorders of immune function.Clinical manifestations are various. In 34 patients,12 died,22 live.The course of disease was 3.4 year.The mean time of treatment with corticosteroids was 2.1 year.The baseline of the age of onset,the age of diagnosis and the levels of Hb,the course of disease,the mean time of treatment with corticosteroids showed no significant differences between the deaths and the survivals(P＞0.05).Conclusion:IPH began in ages between 3-6years mostly.The symptoms are various.The etiology of IPH may be the disorder of immune system.The time of therapy with corticosteroids should be prolonged to 2 years or longer,which may improve the prognosis.Irregular medication may cause poor outcome.