儿童特发性肺含铁血黄素沉着症25例

Clinical study of 25 children with idiopathic pulmonary hemosiderosis

目的 探讨儿童特发性肺含铁血黄素沉着症（IPH）的临床特点、实验室检查、治疗和预后。方法 对1992年4月至2008年11月首都儿科研究所附属儿童医院收治的25例IPH 儿童的临床资料进行分析，对部分患儿进行随访。结果 25例IPH 患儿发病年龄6.5个月～8岁，中位数为4岁，3～6岁儿童所占比例最高（12例，48%），确诊年龄中位数为5.17岁；病程10 d～6年，中位数为1年。咳嗽、面色苍白、发热、咯血是其最常见的临床表现。胸部影像学表现多样，15例高分辨CT中表现为双肺透亮度降低、磨玻璃样改变11例，肺内片絮状阴影9例，网状肺纹理6例，肺内结节影2例。23例患儿在确诊 IPH 前存在误诊，60% 的患儿误诊时间长达1年以上。肾上腺皮质激素治疗可有效改善临床症状。部分患儿多年以后可发生类风湿性关节炎。结论 儿童IPH 临床表现缺乏特异性，容易误诊。胸部影像学检查结合反复痰、胃液及支气管肺泡灌洗液找含铁血黄素细胞有助于IPH 诊断。激素治疗可缓解症状。IPH 与类风湿性关节炎可能存在一定关系。

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 coarse 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. Twenty-three cases were once misdiagnosed and 60% of them were delayed in diagnosis as IPH for more than 1 year. Glucocortieoid therapy was effective in improving symptoms. Some patients suffered from rheumatoid arthritis later in their life. Conclusions The manifestations of IPH in children are nonspeeific, 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.