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多发性肺囊肿患者的Birt-Hogg-Dubé综合征中的肺囊肿特征

Features of Lung Cyst in Birt-Hogg-Dubé Syndrome from Patients with Multiple Lung Cysts.

作者信息

Choi Yong Jun, Park Hye Jung, Kim Chi Young, Jung Bo Mi, Cho Jae Hwa, Byun Min Kwang

机构信息

Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea.

出版信息

Tuberc Respir Dis (Seoul). 2025 Apr;88(2):388-398. doi: 10.4046/trd.2024.0045. Epub 2024 Nov 25.

DOI:10.4046/trd.2024.0045
PMID:39582154
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12010710/
Abstract

BACKGROUND

High-resolution chest computed tomography (CT) is a crucial assessment tool for diagnosing Birt-Hogg-Dubé (BHD) syndrome. This study aimed to analyze differences of lung cysts between BHD and other cystic lung diseases.

METHODS

From January 2020 to December 2022, patients with multiple lung cysts who underwent chest CT at Gangnam Severance Hospital were included.

RESULTS

Over a 3-year period (from January 2020 to December 2022), out of 52,823 patients who underwent a chest CT scan, 301 (0.6%) patients with multiple lung cysts were enrolled in this study. Of enrolled patients, 24 (8.0%) were diagnosed with BHD. In patients with BHD, 95.8% exhibited bilateral cysts, and 83.3% showed basal predominance. The cysts' maximal diameter averaged 32.1 mm (interquartile range, 26.5 to 43.5). Additionally, 95.8% of patients with BHD had diverse cyst sizes and morphologies. Multivariate logistic regression analysis revealed that bilateral cysts (odds ratio [OR], 12.393; 95% confidence interval [CI], 1.613 to 274.682; p=0.038), basal predominance (OR, 8.511; 95% CI, 2.252 to 39.392; p=0.002), maximum diameter (OR, 1.053; 95% CI, 1.009 to 1.108; p=0.032), and diversity of morphology (OR, 19.513; 95% CI, 2.833 to 398.119; p=0.010) were significant factors associated with BHD diagnosis. A multivariate prediction model for BHD diagnosis demonstrated a sensitivity of 95.83%, a specificity of 81.22%, and an area under the receiver operating characteristic curve of 0.951 (95% CI, 0.914 to 0.987).

CONCLUSION

Distinguishing features of lung cysts from other cystic lung diseases include bilateral cysts, basal dominance, large size, and irregular shape.

摘要

背景

高分辨率胸部计算机断层扫描(CT)是诊断Birt-Hogg-Dubé(BHD)综合征的关键评估工具。本研究旨在分析BHD与其他囊性肺疾病之间肺囊肿的差异。

方法

纳入2020年1月至2022年12月在江南Severance医院接受胸部CT检查的多发肺囊肿患者。

结果

在3年期间(2020年1月至2022年12月),在52823例接受胸部CT扫描的患者中,301例(0.6%)多发肺囊肿患者纳入本研究。在纳入的患者中,24例(8.0%)被诊断为BHD。在BHD患者中,95.8%表现为双侧囊肿,83.3%表现为基底优势。囊肿最大直径平均为32.1mm(四分位间距,26.5至43.5)。此外,95.8%的BHD患者囊肿大小和形态多样。多因素logistic回归分析显示,双侧囊肿(比值比[OR],12.393;95%置信区间[CI],1.613至274.682;p=0.038)、基底优势(OR,8.511;95%CI,2.252至39.392;p=0.002)、最大直径(OR,1.053;95%CI,1.009至1.108;p=0.032)和形态多样性(OR,19.513;95%CI,2.833至398.119;p=0.010)是与BHD诊断相关的显著因素。BHD诊断的多因素预测模型显示敏感性为95.83%,特异性为81.22%,受试者工作特征曲线下面积为0.951(95%CI,0.914至0.987)。

结论

与其他囊性肺疾病相比,肺囊肿的鉴别特征包括双侧囊肿、基底优势、大尺寸和不规则形状。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b82d/12010710/1d86d2bf66a9/trd-2024-0045f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b82d/12010710/1d86d2bf66a9/trd-2024-0045f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b82d/12010710/1d86d2bf66a9/trd-2024-0045f1.jpg

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