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儿童朗格汉斯细胞组织细胞增多症:超声在诊断及随访中的价值

Langerhans cell histiocytosis in children: the value of ultrasound in diagnosis and follow-up.

作者信息

Yang Jinjin, Huang Xiaohua, Bao Zhongtao, Xu Jing, Huang Huimei, Huang Hongjie, Chen Ling

机构信息

Department of Ultrasound Medicine, First Affiliated Hospital, Fujian Medical University, Fuzhou, 350005, China.

Department of Ultrasound Medicine, National Regional Medical Center, Binhai Compus of First Affiliated Hospital of Fujian Medical University, Fuzhou, 350212, China.

出版信息

BMC Med Imaging. 2025 Jan 29;25(1):29. doi: 10.1186/s12880-025-01563-x.

DOI:10.1186/s12880-025-01563-x
PMID:39881244
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11776198/
Abstract

BACKGROUND

Langerhans cell histiocytosis (LCH) is a rare disease, most prevalent in children. Ultrasound is a noninvasive, cheap, and widely available technique. However, systematic elucidation of sonographic features of LCH and treatment related follow-up are relatively few, resulting in overall underestimation of the clinical value of ultrasound in diagnosing and monitoring LCH.

OBJECTIVE

This study aimed to observe the sonographic features of Langerhans Cell Histiocytosis (LCH) comparing with other imaging examinations, and to evaluate the changes of ultrasonography in the follow-up of LCH in children.

MATERIALS AND METHODS

Forty-four children (female:male, 19/25; median age, 60 months; range, 8 to 192 months) with LCH were included in this retrospective study. Thirty-one had single-system involvement (SS-LCH), and 13 had multisystem involvement (MS-LCH) among the 44 children. We analyzed the clinical characteristics, ultrasound (US) images, and images from other modalities, including X-ray, computed tomography (CT), and magnetic resonance imaging (MRI). The sonographic characteristics of the various involved organs, particularly bone, thyroid, and liver were analyzed, and the percentage of LCH cases correctly identified by the various imaging modalities were evaluated.

RESULTS

Localized worm-like bone defects solid hypoechoic lesions were found in 38 patients with a total of 43 skeletal lesions, which showed solid hypoechoic lesions on US. Five patients showed hypoechoic or hyperechoic areas in the liver. Two patients showed scattered or diffuse irregular hypoechoic areas in the thyroid. Two patients with skeletal and 1 with thyroid involvement showed smaller lesions and lower blood flow after chemotherapy, and 6 lesions involving the liver resolved or were smaller in US review. The percentage of LCH cases correctly identified of US (65.38%) was higher than that of X-ray (21.05%) (P = 0.026) for skeletal lesions, which was comparable to that of CT and MRI. The overall correctly identified percentage of US for LCH was not significantly different from that of other imaging modalities.

CONCLUSION

LCH can be detected and suspected based on sonographic features. US may be an excellent tool for the diagnosis and follow-up of LCH in children.

摘要

背景

朗格汉斯细胞组织细胞增多症(LCH)是一种罕见疾病,在儿童中最为常见。超声是一种无创、廉价且广泛可用的技术。然而,关于LCH超声特征的系统阐述以及与治疗相关的随访相对较少,导致超声在LCH诊断和监测中的临床价值总体被低估。

目的

本研究旨在观察朗格汉斯细胞组织细胞增多症(LCH)的超声特征,并与其他影像学检查进行比较,同时评估超声在儿童LCH随访中的变化。

材料与方法

本回顾性研究纳入了44例LCH患儿(女性19例,男性25例;中位年龄60个月;范围8至192个月)。44例患儿中,31例为单系统受累(SS-LCH),13例为多系统受累(MS-LCH)。我们分析了临床特征、超声(US)图像以及包括X线、计算机断层扫描(CT)和磁共振成像(MRI)在内的其他影像学检查图像。分析了各个受累器官的超声特征,特别是骨骼、甲状腺和肝脏,并评估了各种影像学检查正确识别LCH病例的百分比。

结果

38例患者共43处骨骼病变发现局限性蠕虫样骨质缺损实性低回声病变,超声显示为实性低回声病变。5例患者肝脏出现低回声或高回声区。2例患者甲状腺出现散在或弥漫性不规则低回声区。2例骨骼受累和1例甲状腺受累患者化疗后病变变小且血流减少,6例肝脏受累病变超声复查显示消退或变小。对于骨骼病变,超声正确识别LCH病例的百分比(65.38%)高于X线(21.05%)(P = 0.026),与CT和MRI相当。超声对LCH的总体正确识别百分比与其他影像学检查无显著差异。

结论

基于超声特征可检测和怀疑LCH。超声可能是儿童LCH诊断和随访的优秀工具。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2e18/11776198/8617f19a0f6e/12880_2025_1563_Fig9_HTML.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2e18/11776198/0f1d44013f87/12880_2025_1563_Fig8_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2e18/11776198/8617f19a0f6e/12880_2025_1563_Fig9_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2e18/11776198/1833ce0de097/12880_2025_1563_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2e18/11776198/5c3a1f07627b/12880_2025_1563_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2e18/11776198/ca4780a85e25/12880_2025_1563_Fig5_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2e18/11776198/19a1a6bc53b4/12880_2025_1563_Fig7_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2e18/11776198/0f1d44013f87/12880_2025_1563_Fig8_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2e18/11776198/8617f19a0f6e/12880_2025_1563_Fig9_HTML.jpg

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