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一名成年患者的特发性肺含铁血黄素沉着症对皮质类固醇疗法反应良好。

Idiopathic pulmonary hemosiderosis in an adult patient responded well to corticosteroid therapy.

作者信息

Deniz Omer, Ongürü Onder, Ors Fatih, Gümüş Seyfettin, Tozkoparan Ergun, Bilgiç Hayati, Ekiz Kudret

机构信息

Department of Chest Disease, Gülhane Military Medicine Academy, Ankara, Turkey.

出版信息

Tuberk Toraks. 2007;55(1):77-82.

Abstract

Idiopathic pulmonary hemosiderosis (IPH) is a very rare disorder of unknown etiology characterized by recurrent or chronic hemorrhage and accumulation of hemosiderin in the lung parenchyma. It is most common in children but can occur in adults. Clinical manifestations of the disease include iron deficiency anemia without any known cause, pulmonary symptoms such as hemoptysis, dyspnea and cough, and parenchymal lesions on chest X-ray. The clinical course of the disease may vary from patient to patient however, in general, the prognosis of the disease is worse. Treatment is symptomatic and supportive. Corticosteroids and other immune suppressive agents were used for the therapy of IPH. Since it is seen rarely in adults and the clinical course of the disease vary from patient to patient we presented an adult male patient with IPH responded well to steroid therapy clinically and radiologically.

摘要

特发性肺含铁血黄素沉着症(IPH)是一种病因不明的极为罕见的疾病,其特征为反复或慢性出血以及含铁血黄素在肺实质内蓄积。它在儿童中最为常见,但也可发生于成人。该疾病的临床表现包括无任何已知病因的缺铁性贫血、咯血、呼吸困难和咳嗽等肺部症状,以及胸部X线显示的实质病变。然而,该疾病的临床病程在不同患者之间可能有所不同,总体而言,疾病的预后较差。治疗以对症和支持治疗为主。皮质类固醇和其他免疫抑制剂曾用于IPH的治疗。由于IPH在成人中较为罕见,且疾病的临床病程因患者而异,我们报告了一名成年男性IPH患者,其对类固醇治疗在临床和影像学上均有良好反应。

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