Emerging clinical picture of lymphangioleiomyomatosis
Emerging clinical picture of lymphangioleiomyomatosis
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DOI：
10.1136/thx.2004.035154
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发表时间：
2005-10-01
期刊：
影响因子：
10
通讯作者：
Johnson, SR
中科院分区：
文献类型：
--
作者：
Cohen, MM;Pollock-BarZiv, S;Johnson, SR
目的：为了提供一个全面的更新的临床图片淋巴管平滑肌瘤病（LAM）使用两个大的患者registrations.Methods：横断面问卷调查，其中包括14个LAM症状，气胸，结节性硬化症（TSC），诊断日期，肺功能测试（PFTs.Results：应答率为70.5%（n = 328）的问题。调查时的平均年龄为46.7岁。调查前1年诊断的女性的平均诊断年龄为42.7岁，10年前诊断的女性的平均诊断年龄为35.8岁。主要症状为呼吸困难（74%）、乏力（72%）、咳嗽（47%）和胸痛（44%）;年轻患者（
Objective: To provide a comprehensive update of the clinical picture of lymphangioleiomyomatosis (LAM) using two large patient registries.Methods: A cross sectional questionnaire survey which included questions on 14 LAM symptoms, pneumothorax, tuberous sclerosis complex (TSC), date of diagnosis, and pulmonary function tests (PFTs).Results: The response rate was 70.5% (n = 328). The mean age at the time of the survey was 46.7 years. The mean age at diagnosis was 42.7 years for women diagnosed 1 year before the survey and 35.8 years for women diagnosed 10 years previously. The main symptoms were dyspnoea (74%), fatigue (72%), cough (47%) and chest pain (44%); younger patients (