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[一名16岁女性的特发性肺含铁血黄素沉着症]

[Idiopathic pulmonary hemosiderosis in a 16-year old female].

作者信息

Pedersen F M, Milman N, Jacobsen G K

机构信息

Medicinsk-lungemedicinsk afdeling Y, Amtssygehuset i Gentofte.

出版信息

Ugeskr Laeger. 1996 Feb 12;158(7):921-2.

PMID:8638331
Abstract

Idiopathic pulmonary haemosiderosis (IPH) is a rare disease of unknown aetiology. The clinical and paraclinical findings consist of recurrent haemoptysis, pulmonary infiltrates and iron deficiency anaemia. Examination of sputum or bronchoalveolar lavage fluid obtained at fiberoptic bronchoscopy discloses large numbers of haemosiderin-laden alveolar macrophages. Pulmonary interstitial fibrosis may develop. As pulmonary haemosiderosis can be observed in association with several diseases, IPH is basically a diagnosis made by exclusion of other causes. The treatment consists of immunosuppression with steroids and cytotoxic drugs.

摘要

特发性肺含铁血黄素沉着症(IPH)是一种病因不明的罕见疾病。临床和辅助检查结果包括反复咯血、肺部浸润和缺铁性贫血。对纤维支气管镜检查时获取的痰液或支气管肺泡灌洗液进行检查,可发现大量含铁血黄素的肺泡巨噬细胞。可能会发展为肺间质纤维化。由于肺含铁血黄素沉着症可与多种疾病相关,IPH基本上是通过排除其他病因后做出的诊断。治疗包括使用类固醇和细胞毒性药物进行免疫抑制。

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