Uncommon radiologic computed tomography appearances of the chest in patients with lymphangioleiomyomatosis.
Uncommon radiologic computed tomography appearances of the chest in patients with lymphangioleiomyomatosis.
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DOI：
10.1038/s41598-021-85999-5
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发表时间：
2021-03-30
影响因子：
4.6
通讯作者：
Seyama K
中科院分区：
文献类型：
--
作者：
Sekimoto Y;Suzuki K;Okura M;Hayashi T;Ebana H;Kumasaka T;Mitani K;Nishino K;Okamoto S;Kobayashi E;Takahashi K;Seyama K
淋巴管平滑肌瘤病（LAM）是一种罕见的破坏性肺部疾病，其特征是多发性薄壁肺囊肿。目前提出的诊断算法强调高分辨率计算机断层扫描 (HRCT) 上的特征性囊性外观，因此不常见的 HRCT 外观对建立正确的 LAM 诊断提出了挑战。本研究的目的是收集不常见的胸部 HRCT 表现，确定结节性硬化症 (TSC) 相关 LAM (TSC-LAM) 和散发性 LAM (S-LAM) 患者的频率。对转诊至我们医院的 311 名女性进行回顾性评估，其中包括 272 名 S-LAM 患者（平均年龄 39.2 岁）和 39 名 TSC-LAM 患者（平均年龄 38.3 岁）。我们发现两种类型的放射学结果可能使 HRCT 囊肿外观不典型：囊肿本身的特征和除囊肿之外的罕见发现。我们发现，大约 80% 的 LAM 患者，无论是 TSC 相关的还是散发的，都表现出典型的 HRCT 表现，伴有轻度至重度囊性破坏。其余 20% 的囊肿外观表现出不寻常的特征，以及囊肿以外的其他发现：前者包括大囊肿、壁增厚和形状不规则，而后者包括毛玻璃衰减和弥漫性非钙化结节。重要的是要了解导致 HRCT 囊性外观非典型 LAM 的各种放射学结果。
Lymphangioleiomyomatosis (LAM) is a rare destructive lung disease characterized by multiple thin-walled pulmonary cysts. The currently proposed diagnostic algorithm emphasizes the characteristic cystic appearance on high-resolution computed tomography (HRCT) so uncommon HRCT appearances present challenges to establishing the proper LAM diagnosis. The objective of this study is to accrue uncommon chest HRCT appearances, determine frequencies in both tuberous sclerosis complex (TSC)-associated LAM (TSC-LAM) and sporadic LAM (S-LAM) patients. 311 females referred to our hospital, including 272 S-LAM patients (mean age 39.2 years) and 39 TSC-LAM patients (mean age 38.3 years), were retrospectively evaluated. We found 2 types of radiologic findings likely to make HRCT cyst appearance atypical: characteristics of the cyst itself and uncommon findings in addition to cysts. We found that approximately 80% of LAM patients, whether TSC-associated or sporadic, showed typical HRCT appearance with mild to severe cystic destruction. The remaining 20% displayed unusual profiles in cyst appearance as well as additional findings aside from cyst: the former includes large cyst, thickened walls, and irregularly shaped whereas the latter includes ground glass attenuation and diffuse noncalcified nodules. It is important to be aware of various radiologic findings that make HRCT cystic appearance atypical of LAM.
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结节性硬化症复杂诊断标准更新：2012年iinternation Tuberous硬化症复杂共识会议的建议。
影响因子：
3.8
作者：
Northrup H;Krueger DA;International Tuberous Sclerosis Complex Consensus Group
通讯作者：
International Tuberous Sclerosis Complex Consensus Group
DOI：
10.1164/ajrccm.164.4.2011025
发表时间：
2001-08-15
影响因子：
24.7
作者：
Franz, DN;Brody, A;McCormack, FX
通讯作者：
McCormack, FX
DOI：
10.1164/rccm.201709-1965st
发表时间：
2017-11-15
影响因子：
24.7
作者：
Gupta, Nishant;Finlay, Geraldine A.;McCormack, Francis X.
通讯作者：
McCormack, Francis X.
影响因子：
1.4
作者：
Seyama, Kuniaki;Kumasaka, Toshio;Sato, Teruhiko
通讯作者：
Sato, Teruhiko
影响因子：
4.5
作者：
Giannikou K;Malinowska IA;Pugh TJ;Yan R;Tseng YY;Oh C;Kim J;Tyburczy ME;Chekaluk Y;Liu Y;Alesi N;Finlay GA;Wu CL;Signoretti S;Meyerson M;Getz G;Boehm JS;Henske EP;Kwiatkowski DJ
通讯作者：
Kwiatkowski DJ