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以自发性气胸为表现的淋巴管平滑肌瘤病病例报告。

A case report of lymphangioleiomyomatosis presenting as spontaneous pneumothorax.

作者信息

Riojas Ramon A, Bahr Brady A, Thomas David B, Perciballi John, Noyes Lachland

机构信息

81st Medical Group, 301 Fisher Street, Keesler AFB, MS 39534, USA.

出版信息

Mil Med. 2012 Apr;177(4):477-80. doi: 10.7205/milmed-d-11-00333.

Abstract

Spontaneous pneumothorax is a commonly encountered problem in the Emergency Department. Patients are often treated without further investigation for an underlying etiology. We present a patient who was unable to completely resolve a pneumothorax and was found to have lymphangioleiomyomatosis (LAM), a rare cystic lung disease. In the past, LAM was difficult to diagnose and had a mortality of 100% after 10 years, but now there is a 71% survival after 10 years. Recent research has led to increased characterization of the pathology and radiographic findings. This article briefly presents the case and discusses the etiology, diagnosis, and treatment of LAM.

摘要

自发性气胸是急诊科常见的问题。患者通常在未对潜在病因进行进一步检查的情况下就接受治疗。我们报告了一名患者,其气胸无法完全缓解,最终被诊断为淋巴管平滑肌瘤病(LAM),这是一种罕见的囊性肺部疾病。过去,LAM很难诊断,10年后死亡率为100%,但现在10年后的生存率为71%。最近的研究使得对其病理和影像学表现的认识有所增加。本文简要介绍该病例,并讨论LAM的病因、诊断和治疗。

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