儿童特发性肺含铁血黄素沉着症临床特点及随访研究  

Clinical follow-up studies of pediatric idiopathic pulmonary hemosiderosis

作　　者：求伟玲[1] 陈志敏[1] 

机构地区：[1]浙江大学医学院附属儿童医院呼吸科,杭州310003

出　　处：《中华实用儿科临床杂志》2013年第4期285-287,共3页Chinese Journal of Applied Clinical Pediatrics

摘　　要：目的总结儿童特发性肺含铁血黄色沉着症（IPH）临床特点及预后不良的危险因素。方法回顾性分析本院41例IPH患儿的临床资料，并对其中22例随访患儿的预后进行随访研究。根据随访结果分为存活组及死亡组，并将2组相关临床因素进行统计分析。结果IPH患儿41例，男14例，女27例；发病年龄中位数3．8岁，确诊年龄中位数4．6岁。临床主要表现：面色苍白34例（82．9％），咳嗽27例（65．8％），咯血16例（39．0％），乏力13例（31．7％），发热11例（26．8％）。首发表现：面色苍白27例（65．9％），咳嗽13例（31．7％），咯血11例（26．8％）。辅助检查呈现小细胞低色素贫血（Hb平均值为65．2g／L），胸部x线片异常40例（97．6％）。采用激素治疗40例，治疗后症状均有缓解。随访22例，随访时间6个月～9年，6例（27．3％）死亡，16例（72．7％）存活，死亡组与存活组患儿在发病年龄、性别、确诊年龄、入院时Hb水平、有无咯血及是否需吸氧上差异均无统计学意义（P均〉0．05），死亡组胆红素升高者多于存活组，差异有统计学意义（P〈0．05）。结论IPH临床表现多样，误诊率高，对中度以上的小细胞低色素贫血应常规行胸片检查，激素治疗可使部分患儿病情稳定，但病死率仍高，胆红素升高可能与预后不良有关。Objective To describe the clinical characteristics of idiopathic pulmonary hemosiderosis （IPH）, and to investigate the possible risk factors for poor prognosis. Methods The clinical data of 41 patients with IPH were retrospectively analyzed,22 cases were divided into survival group and death group according to the follow-up data and prognosis, and the related clinical factors in 2 groups were statistically analyzed. Results Of the 41 patients with IPH, 14 cases were male,27 cases female. The median age of onset was 3.8 years, and the diagnosed median age was 4.6 years. The common clinical features of the 41 children with IPH included pale, cough, hemoptysis, fever, and fatigue, in 34 cases （82.9%） ,27 cases （65.8%）, 16 cases （39.0%）, 13 cases （31.7%） and 11 cases （26.8%） children, respectively. Initial symptoms included pale, cough, hemoptysis, in 27 cases （65.9%）, 13 cases （31.7 % ）, and 11 cases （26.8%） children, respectively. Accessory examinations revealed microcytic hypochromic anemia （average hemoglobin concentration was 65.2 g/L）, and 40 patients （97.6%）had an abnormal chest X-ray. Forty patients were treated with glucocorticoids, and all of them had symptom remission. Twenty-two patients were followed up for 6 months to 9 years, and 6 cases （27.3%）died during follow-up and 16 cases （72.7%） still alive. There were no statistically significant differences between death group and survival group as to age of onset, gender distribution, age on diagnosis, degree of anemia, and clinical features （ all P 〉 0.05 ）. The incidence of jaundice was significantly different between the 2 groups （ P 〈 0.05 ）. Conclusions IPH has diverse clinical manifestations and a high rate of misdiagnosis. A routine chest X- ray film should be taken for patients with moderate to severe microcytic hypochromic anemia. The conditions of some pediatric patients can be stabilized with glucocorticoids treatment, but the associated mortality rate remains high. History of jaundic

关 键 词：特发性肺含铁血黄素沉着症 预后 儿童 

分 类 号：R72[医药卫生—儿科]