Clinical study of 25 children with idiopathic pulmonary hemosiderosis / 中华实用儿科临床杂志

ABSTRACT

Objective To analyze the clinical features,laboratory results,treatment and prognosis of children with idiopathic pulmonary hemosiderosis (IPH).Methods The documents of 25 children with IPH,who were hospitalized in Children's Hospital Affiliated to Capital Institute of Pediatrics from Apr.1992 to Nov.2008 were reviewed.Then some of the patients were followed up.Results The median age of onset was 4 years old (6.5 months to 8 years old).Those patients aged from 3 to 6 years old ranked the first as 48％ (12 cases).The median age at diagnosis was 5.17 years old.The course of desease was between 10 days and 6 years,the median course was 1 year.Cough,pallor,fever and hemoptysis were the common clinical features of IPH patients.The chest radiological patterns for IPH were diverse.The common features of high resolution CT scan in 15 patients included declined transparency and ground glass shadows in 11 cases,cloudy patchy infiltrate in 9 cases,reticular changes in 6 cases,and nodular changes in 2 children.Twentythree cases were once misdiagnosed and 60％ of them were delayed in diagnosis as IPH for more than 1 year.Glucocorticoid therapy was effective in improving symptoms.Some patients suffered from rheumatoid arthritis later in their life.Conclusions The manifestations of IPH in children are nonspecific,therefore it is easily to be misdiagnosed.Combined chest radiographic presentations with repeatedly looking for hemosiderin-laden macrophages in sputum,gastric aspirate or bronchoalveolar lavage fluid are helpful in diagnosis.Glucocorticoid therapy can control the symptoms.Some relationships may exist between IPH and rheumatoid arthritis.