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目的总结儿童特发性肺含铁血黄素沉着症的临床特点并讨论诊治及预后,减少误诊及漏诊。方法对确诊特发性肺含铁血黄素沉着症患儿12例进行回顾性分析并对所有患儿随诊。结果 12例患儿年龄10月至8岁之间。临床主要表现为咳嗽10例(83%),面色苍白8例(67%),咯血或痰中带血5例(42%),发热3例(25%);主要体征为呼吸音增粗2例(16%),肺部湿啰音5例(42%)。免疫功能呈现不同程度的异常。随访12例中2例(17%)死亡(死亡原因为肺内大出血),10例(83%)存活。死亡组与存活组患儿在发病年龄、血红蛋白、用药疗程、性别分布之间差异无统计学意义(P>0.05)。结论特发性肺含铁血黄素沉着症好发于1～7岁儿童,误诊时间较长,可出现免疫功能紊乱,临床表现多样,部分患儿经长疗程激素治疗后可处于较稳定的状态。
Objective To summarize the clinical features of idiopathic pulmonary hemosiderosis in children and to discuss the diagnosis, treatment and prognosis, to reduce misdiagnosis and missed diagnosis. Methods A retrospective analysis of 12 children with idiopathic pulmonary hemosiderosis was performed and all children were followed up. Results 12 cases of children aged 10 months to 8 years old. The main clinical manifestations were cough in 10 cases (83%), pale pale in 8 cases (67%), hemoptysis or sputum in 5 cases (42%), fever in 3 cases (25%); Cases (16%), lung wet rales in 5 cases (42%). Immune function showed varying degrees of abnormalities. Two of the 12 patients (17%) were followed up (bleeding due to intrapulmonary hemorrhage) and 10 (83%) survived. There was no significant difference in the age of onset, hemoglobin, medication course and sex distribution between the death group and the survival group (P> 0.05). Conclusions Idiopathic pulmonary hemosiderosis is common in children aged 1 to 7 years. Misdiagnosis time is longer, immune dysfunction may occur, and clinical manifestations are diverse. Some children may be in a stable state after prolonged course of hormone therapy .