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采用扩散加权磁共振成像对Ⅰ型戈谢病患儿的肝脏和脾脏进行评估。

Assessment of the liver and spleen in children with Gaucher disease type I with diffusion-weighted MR imaging.

作者信息

Razek Ahmed Abdel Khalek Abdel, Abdalla Ahmed, Barakat Tarik, El-Taher Heba, Ali Khadiga

机构信息

Department of Diagnostic Radiology, Mansoura Faculty of Medicine, Mansoura, Egypt.

Gastroenterology and Hepatology Unit of Mansoura Children Hospital, Mansoura Faculty of Medicine, Mansoura, Egypt.

出版信息

Blood Cells Mol Dis. 2018 Feb;68:139-142. doi: 10.1016/j.bcmd.2016.12.004. Epub 2016 Dec 20.

Abstract

PURPOSE

To assess hepatic and splenic apparent diffusion coefficient (ADC) in children with Gaucher disease type I with diffusion-weighted MR imaging and to correlate hepatic and splenic ADC with parameters of disease severity.

SUBJECTS AND METHODS

Prospective study was conducted upon 25 children (11 treated and 14 untreated) with Gaucher disease and 12 age and sex matched control children. They underwent diffusion-weighted MR imaging of abdomen. Hepatic and splenic ADC and volume were calculated.

RESULTS

There was statistically difference in hepatic and splenic apparent diffusion coefficient (P=0.001) between patients and controls. The cutoff ADC of liver and spleen used to differentiate patients from controls were 0.47 and 0.39×10mm with area and curve of 0.947 and 0.886 respectively. There was significant difference in hepatic and splenic ADC between untreated and treated patients (P=0.003 and 0.001). Hepatic ADC correlated with splenic volume (r=-0.721), hepatic volume (r=-0.555) and chitotriosidase (r=-0.413). Splenic ADC correlated with splenic volume (r=-0.652), hepatic volume (r=-0.544) and chitotriosidase (r=-0.355).

CONCLUSION

Hepatic and splenic ADC can detect hepatic and splenic infiltration in Gaucher disease and correlated with some parameters of disease severity.

摘要

目的

利用磁共振扩散加权成像评估Ⅰ型戈谢病患儿肝脏和脾脏的表观扩散系数(ADC),并将肝脏和脾脏的ADC与疾病严重程度参数进行关联。

对象与方法

对25例戈谢病患儿(11例接受治疗,14例未接受治疗)和12例年龄及性别匹配的对照儿童进行前瞻性研究。他们接受了腹部磁共振扩散加权成像检查。计算肝脏和脾脏的ADC及体积。

结果

患者与对照组之间肝脏和脾脏的表观扩散系数存在统计学差异(P = 0.001)。用于区分患者与对照组的肝脏和脾脏ADC临界值分别为0.47和0.39×10⁻³mm²/s,曲线下面积分别为0.947和0.886。未治疗患者与治疗患者之间肝脏和脾脏的ADC存在显著差异(P = 0.003和0.001)。肝脏ADC与脾脏体积(r = -0.721)、肝脏体积(r = -0.555)和壳三糖苷酶(r = -0.413)相关。脾脏ADC与脾脏体积(r = -0.652)、肝脏体积(r = -0.544)和壳三糖苷酶(r = -0.355)相关。

结论

肝脏和脾脏的ADC可检测戈谢病中的肝脏和脾脏浸润,并与疾病严重程度的一些参数相关。

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