Radiological evidence of lymphangioleiomyomatosis in female and male patients with tuberous sclerosis complex

Radiological evidence of lymphangioleiomyomatosis in female and male patients with tuberous sclerosis complex
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DOI:
10.1016/j.crad.2011.02.009
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发表时间:
2011-07-01
期刊:
影响因子:
2.6
通讯作者:
Prokop, M.
Prokop, M.
中科院分区:
医学4区
文献类型:
--
作者:
Adriaensen, M. E. A. P. M.;Schaefer-Prokop, C. M.;Prokop, M.

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目的:研究已知结节性硬化症(TSC)的成人患者中典型淋巴管肌瘤病(LAM)肺囊肿的性别特异性患病率。材料和方法:对206例成人TSC患者进行回顾性横断面研究。获得了机构审查委员会的批准,放弃了患者知情同意。1996至2006年间,患者常规接受腹部CL‘扫描。全部186例患者(平均年龄38岁;年龄范围19-72岁;91例(49%)男性患者)在计算机断层扫描(CT)上至少显示了肺底。复习影像以了解肺内薄壁囊肿的存在。采用描述性统计、两样本t检验比较均数、卡方检验比较比例。结果:186例患者中52例(28%)CT显示肺底薄壁囊肿。大小从直径2毫米到2厘米以上不等。95例女性患者中有40例(42%)检出肺囊肿,91例男性患者中有12例(13%)检出肺囊肿(p<0.001)。总体而言,女性的囊肿比男性的更大、更多。仅有4名女性和2名男性有轻微的囊性改变,3名女性和7名男性有中度的囊性改变,但相当大的囊性改变几乎仅见于女性(33名女性和3名男性)。结论:在186例结节硬化症患者中,28%的患者CT显示肺基底部有薄壁肺囊肿。女性患者比男性患者受影响更大。(C)2011年皇家放射科医师学会。爱思唯尔有限公司出版。保留所有权利。
AIM: To determine the gender-specific prevalence of pulmonary cysts typical for lymphangioleiomyomatosis (LAM) in adult patients with known tuberous sclerosis complex (TSC).MATERIALS AND METHODS: A retrospective, cross-sectional study in a cohort of 206 adult TSC patients was performed. Institutional review board approval was obtained, and patient informed consent was waived. Patients had routinely undergone abdominal Cl' scanning between 1996 and 2006. All 186 patients (mean age 38 years; range 19-72 years; 91(49%) male patients) in whom at least the lung bases were depicted on computed tomography (CT) were included. Images were reviewed for the presence of pulmonary thin-walled cysts. Descriptive statistics, two sample t-test to compare means, and chi(2)-test to compare proportions were applied.RESULTS: CT demonstrated pulmonary thin-walled cysts in the lung bases in 52 (28%) of 186 patients. Size varied from 2 mm in diameter to more than 2 cm. Pulmonary cysts were detected in 40(42%) of 95 female patients and in 12(13%) of 91 male patients (p < 0.001). In general, cysts were larger and more numerous in women than in men. Only minimal cystic changes were found in four women and two men, moderate cystic changes were seen in three women and seven men, but considerable cystic changes were seen almost exclusively in women (33 women versus three men).CONCLUSION: CT demonstrated thin-walled pulmonary cysts in the lung bases in 28% of 186 included patients with tuberous sclerosis complex. Female patients were more affected than male patients. (C) 2011 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.