High frequency of pulmonary lymphangioleiomyomatosis in women with tuberous sclerosis complex
High frequency of pulmonary lymphangioleiomyomatosis in women with tuberous sclerosis complex
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DOI：
10.4065/75.6.591
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发表时间：
2000-06-01
影响因子：
8.9
通讯作者：
Ryu, JH
中科院分区：
文献类型：
--
作者：
Costello, LC;Hartman, TE;Ryu, JH
目的：为了确定肺淋巴管平滑肌瘤病（LAM）（一种罕见的囊性肺疾病，几乎仅发生在育龄妇女中）在结节性硬化症（TSC）（一种遗传性多器官错构瘤病）妇女中的频率，患者和方法：在这项回顾性队列研究中，审查了78名确诊TSC的女性的病历，并记录了相关信息，包括人口统计学数据，临床表现、肺活组织检查和尸检结果以及胸部和腹部影像学检查结果。所有可用的计算机断层扫描（CT）扫描的胸部和腹部review.Results：78名妇女明确TSC看到1977年至1998年，20（26%）有证据的LAM。7例经手术肺活检或尸检证实为肺部疾病。在另外13例患者中观察到LAM的特征性CT表现。12这20例TSC相关的LAM有呼吸道症状，包括劳力性呼吸短促和自发性气胸，最终导致他们的肺部diagnosis.Conclusions：肺受累（LAM）的频率在TSC的妇女大大高于以前怀疑，甚至可能高于在这项回顾性研究中报告。这些发现支持了对所有TSC女性进行胸部CT筛查的建议。
Objective: To determine the frequency of pulmonary lymphangioleiomyomatosis (LAM), a rare cystic lung disorder that occurs almost exclusively in women of reproductive age, in women with tuberous sclerosis complex (TSC), an inheritable multiorgan hamartomatosis,Patients and Methods: In this retrospective cohort study, the medical records of 78 women with definite TSC were reviewed, and pertinent information was recorded, including the demographic data, clinical manifestations, results of lung biopsies and autopsies, and findings on imaging studies of the chest and abdomen. All available computed tomographic (CT) scans of the chest and abdomen were reviewed.Results: Of 78 women with definite TSC seen from 1977 to 1998, 20 (26%) had evidence of LAM. Surgical lung biopsy or autopsy in 7 patients confirmed the diagnosis of their lung disease. Characteristic CT findings of LAM were noted in 13 additional patients. Twelve of these 20 patients with TSC-associated LAM had respiratory symptoms, including exertional shortness of breath and spontaneous pneumothorax, that eventually led to their pulmonary diagnosis.Conclusions: The frequency of Lung involvement (LAM) in women with TSC is substantially higher than previously suspected and may be even higher than reported in this retrospective study. These findings support the recommendation for a screening CT of the chest for all women with TSC.