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[结节性硬化综合征伴淋巴管平滑肌瘤病患者的细菌学确诊肺结核]

[Bacteriologically confirmed pulmonary tuberculosis in a patient with lymphangioleiomyomatosis accompanying tuberous sclerosis syndrome].

作者信息

Lewandowska Katarzyna, Oniszh Karina, Augustynowicz-Kopeć Ewa, Radwan-Röhrenschef Piotr, Kuś Jan

机构信息

I Klinika Chorób Płuc, Instytut Gruźlicy i Chorób Płuc, ul. Płocka 26, Warsaw.

出版信息

Pneumonol Alergol Pol. 2011;79(4):309-14.

PMID:21678282
Abstract

Lymphangioleiomyomatosis (LAM) is a rare disease of unknown origin, that may be sporadic or develop in the course of tuberous sclerosis (TS). Patients do not present immune deficiency, but structural changes in the lung parenchyma (cysts) may encourage various infections, for example tuberculosis. Radiologic findings are often difficult to interpret, because of changes related to LAM itself. We present a young women with a history of TS and LAM, in whom protracting respiratory tract infection was finally diagnosed as tuberculosis. Initial diagnosis was based primarily on clinical signs and symptoms and treatment was started despite the negative result of the sputum microscopy for acid fast bacilli. In the course of treatment the diagnosis was supported by positive tuberculin skin test, interferon-gamma release assay and genetic test for M. tuberculosis in bronchoalveolar lavage fluid, and finally, positive sputum culture in liquid medium.

摘要

淋巴管平滑肌瘤病(LAM)是一种病因不明的罕见疾病,可散发或在结节性硬化症(TS)病程中发生。患者不存在免疫缺陷,但肺实质的结构改变(囊肿)可能会引发各种感染,例如肺结核。由于与LAM本身相关的变化,影像学表现往往难以解读。我们报告一位有TS和LAM病史的年轻女性,其迁延不愈的呼吸道感染最终被诊断为肺结核。初始诊断主要基于临床症状和体征,尽管痰涂片抗酸杆菌检测结果为阴性,但仍开始了治疗。在治疗过程中,结核菌素皮肤试验、干扰素-γ释放试验以及支气管肺泡灌洗液中结核分枝杆菌的基因检测结果均为阳性,最终液体培养基痰培养结果也呈阳性,从而支持了该诊断。

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Pneumonol Alergol Pol. 2011;79(4):309-14.
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Lymphangioleiomyomatosis (LAM) presenting as recurrent pneumothorax in an infant with tuberous sclerosis: treated successfully with sirolimus.淋巴管平滑肌瘤病(LAM)在一名患有结节性硬化症的婴儿中表现为复发性气胸:西罗莫司治疗成功。
BMJ Case Rep. 2018 Nov 8;2018:bcr-2018-226244. doi: 10.1136/bcr-2018-226244.

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