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[淋巴管平滑肌瘤病与结节性硬化症:偶然关联还是因果关系?]

[Lymphangioleiomyomatosis and tuberous sclerosis: a casual association or a causative one?].

作者信息

Lana R, Sánchez-Alarcos J M, Martínez-Cruz R, Calle M, Alvarez-Sala J L

机构信息

Servicio de Medicina Interna I, Hospital Clínico San Carlos, Universidad Complutense, Madrid.

出版信息

Arch Bronconeumol. 1998 Oct;34(9):463-5. doi: 10.1016/s0300-2896(15)30373-2.

Abstract

Lymphangioleiomyomatosis (LAM) is a rare lung disease that mainly affects women of childbearing age. Pulmonary disease that is indistinguishable from LAM appears in 1% of patients with tuberous sclerosis (TS). At present we can not say whether we are looking at an association of two rare diseases or at a single disease with different clinical profiles. To date, open lung biopsy has been required for definitive diagnosis. We report the case of a women with TS and LAM in whom a diagnosis of pulmonary involvement was based on clinical signs and high resolution computerized tomography of the chest.

摘要

淋巴管平滑肌瘤病(LAM)是一种罕见的肺部疾病,主要影响育龄女性。1%的结节性硬化症(TS)患者会出现与LAM难以区分的肺部疾病。目前,我们尚不能确定这是两种罕见疾病的关联,还是一种具有不同临床特征的单一疾病。迄今为止,明确诊断需要进行开胸肺活检。我们报告了一例患有TS和LAM的女性病例,其肺部受累的诊断基于临床体征和胸部高分辨率计算机断层扫描。

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