肺含铁血黄素沉着症26例的临床特点和预后分析

邱奕宁; 郝琎琎; 赵琳; 余慧; 肖燕; 张志泉; 金润铭

doi:10.13201/j.issn.1009-5918.2012.06.024

肺含铁血黄素沉着症26例的临床特点和预后分析

- 华中科技大学同济医学院附属协和医院儿科(武汉, 430022)

详细信息

通讯作者: 金润铭,E-mail:erke_xiehe@yahoo.com.cn

中图分类号: R72

收稿日期: 2012-04-26

Retrospective analysis of clinical characteristic and factors affecting prognosis of 26children with idiopathic pulmonary hemosiderosis

- Department of Pediatrics, Union Hospital Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, China

More Information

Corresponding author: JIN Runming, E-mail: erke_xiehe@yahoo.com.cn

Received Date: 26 April 2012

摘要

摘要: 目的: 探讨儿童特发性肺含铁血黄素沉着症(Idiopathic Pulmonary Hemosiderosis,IPH)的临床特点、诊断、治疗以及预后的危险因素。方法: 对我院2004-2011年收治的26例特发性肺含铁血黄素沉着症患儿资料进行临床回顾性分析及追踪随访。结果: 临床症状主要为面色苍白20例(76.9%),咳嗽或咳喘22例(84.6%),发热12例(46.1%),咯血或痰中带血10例(38.5%)。痰或胃液中可找到含铁血黄素细胞(88.4%)。从出现症状到确诊的平均时间为18.9月。26例中死亡3例,好转或无症状17例,但其中8例存在病情反复,6例失访。从发病到确诊的时间长短是预后危险因素。结论: IPH临床表现多样,容易误诊。激素冲击治疗或大剂量丙种球蛋白联合泼尼松口服可较好地控制IPH急性期病情。通过长期激素治疗可维持长期生存,但部分患儿仍反复发作。及时诊断,及早治疗,有助于控制病情,改善预后。
- 特发性肺含铁血黄素沉着症 /
- 儿童 /
- 临床特点 /
- 丙种球蛋白
Abstract: Objective: To investigate the clinical characteristic, diagnosis, treatment and possible factors affecting prognosis of pediatric idiopathic pulmonary hemosiderosis (IPH).Method: The data of clinical manifestation, laboratory data, treatment and outcomes of 26 children with IPH admitted in our hospital during the past 7 years were retrospectively analyzed.Result: The most important clinical presentation included pale (76.9%), cough (84.6%), fever (46.1%), hemoptysis or bloody sputum (38.5%). The hemosiderin cells could be found in sputum or gastric juice (88.4%). The mean time from onset to final diagnosis was 18.9 months. 3 patients died, 17 patients got symptoms relieved after treatment, but 8 patients of them had recurrent attacks. 6 patients missed during follow up. The duration since onset to diagnosis was independent adverse prognostic factor.Conclusion: Pediatric IPH had diverse clinical manifestations and easy to be misdiagonsed. Using hormone pulse therapy or combined immunotherapy (high dose intravenous immunoglobulin and oral prednisone) could control the acute seizure effectively. Most of patients could have long term survival by taking hormone, but some of them still had recurrent attacks. To diagnosis and start treat in time would be good to prognosis.
- idiopathic pulmonary hemosiderosis /
- children /
- clinical characteristics /
- immunoglobulin

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