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Xiuxiu Wu, Wenshuai Xu, Jun Wang, Xinlun Tian, Zhuang Tian, Kaifeng Xu
《医学前沿（英文）》 2019年 第13卷 第2期 页码 259-266 doi: 10.1007/s11684-018-0634-z
关键词： lymphangioleiomyomatosis pulmonary hypertension pulmonary function hypoxemia sirolimus
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《医学前沿（英文）》 2013年 第7卷 第3期 页码 316-327 doi: 10.1007/s11684-013-0269-z
Diffuse cystic lung diseases are uncommon but can present a diagnostic challenge because increasing number of diseases have been associated with this presentation. Cyst in the lung is defined as a round parenchymal lucency with a well-defined thin wall (<2 mm thickness). Focal or multifocal cystic lesions include blebs, bullae, pneumatoceles, congenital cystic lesions, traumatic lesions, and several infectious processes such as coccidioidomycosis, Pneumocystis jiroveci pneumonia, and hydatid disease. “Diffuse” distribution in the lung implies involvement of all lobes. Diffuse lung involvement with cystic lesions can be seen in pulmonary lymphangioleiomyomatosis, pulmonary Langerhans’ cell histiocytosis, lymphoid interstitial pneumonia, Birt-Hogg-Dubé syndrome, amyloidosis, light chain deposition disease, honeycomb lung associated with advanced fibrosis, and several other rare causes including metastatic disease. High-resolution computed tomography of the chest helps define morphologic features of the lung lesions as well as their distribution and associated features such as intrathoracic lymphadenopathy. Correlating the tempo of the disease process and clinical context with chest imaging findings serve as important clues to defining the underlying nature of the cystic lung disease and guide diagnostic evaluation as well as management.
关键词： cyst lung disease interstitial lung disease lymphangioleiomyomatosis Langerhans’ cell histiocytosis Birt-Hogg-Dubé syndrome
Utility of transbronchial biopsy in the diagnosis of lymphangioleiomyomatosis
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《医学前沿（英文）》 2012年 第6卷 第4期 页码 395-405 doi: 10.1007/s11684-012-0231-5
Pulmonary lymphangioleiomyomatosis (LAM) is a rare cystic lung disease that targets women during their reproductive years. A confident diagnosis can often be based on clinical grounds, but diagnostic certainty requires pathological analysis. Although surgical lung biopsy is considered the gold standard for obtaining tissue in patients with diffuse lung disease, it is also associated with higher morbidity and mortality than alternative, less invasive techniques. The objective of our study was to examine the utility of transbronchial biopsy in the diagnosis of LAM. We conducted two online surveys of over 1 000 LAM patients registered with the LAM Foundation who were accessible by email. Transbronchial biopsy specimens were subsequently collected and reviewed by an expert pathologist to validate the diagnosis. We found that transbronchial biopsy has a yield of approximately 60% in patients with LAM. We conclude that transbronchial biopsy may be a safe and effective method for establishing the diagnosis of LAM, obviating the need for surgical lung biopsy in more than half of LAM patients.
关键词： lymphangioleiomyomatosis lymphangiomyomatosis multicystic lung disease diffuse cystic lung disease transbronchial biopsy perivascular epithelioid cell tumor (PEComa) HMB-45
标题 作者 时间 类型 操作
Clinical characteristics in lymphangioleiomyomatosis-related pulmonary hypertension: an observation on
Xiuxiu Wu, Wenshuai Xu, Jun Wang, Xinlun Tian, Zhuang Tian, Kaifeng Xu
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