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戈谢病:46例患者的腹部磁共振成像表现

Gaucher disease: abdominal MR imaging findings in 46 patients.

作者信息

Hill S C, Damaska B M, Ling A, Patterson K, Di Bisceglie A M, Brady R O, Barton N W

机构信息

Department of Diagnostic Radiology, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 20892.

出版信息

Radiology. 1992 Aug;184(2):561-6. doi: 10.1148/radiology.184.2.1620865.

DOI:10.1148/radiology.184.2.1620865
PMID:1620865
Abstract

Abdominal magnetic resonance imaging findings were reviewed in 46 patients with Gaucher disease. All patients had hepatosplenomegaly at the time of initial imaging. Splenic nodules were present in 14 patients (30%) and varied in signal intensity. These nodules were isointense on T1-weighted and hypointense on T2-weighted images. Splenic infarcts were seen in 15 patients (33%), and four of these patients (9%) also had subcapsular fluid collections. Both nodules and infarcts were present in the spleen in four patients (9%). Pathologic correlation was performed with specimens from two patients who underwent partial splenectomy. Focal areas of abnormal signal intensity were noted in the liver in nine patients (20%). They were either stellate or segmental, and may represent fibrotic septa with ischemic changes associated with aggregates of Gaucher cells. No changes were noted in the kidneys or abdominal lymph nodes.

摘要

对46例戈谢病患者的腹部磁共振成像结果进行了回顾。所有患者在初次成像时均有肝脾肿大。14例患者(30%)出现脾结节,信号强度各异。这些结节在T1加权像上呈等信号,在T2加权像上呈低信号。15例患者(33%)出现脾梗死,其中4例患者(9%)还伴有脾包膜下积液。4例患者(9%)的脾脏同时存在结节和梗死。对2例行部分脾切除术患者的标本进行了病理对照。9例患者(20%)的肝脏发现局灶性异常信号强度区域。它们呈星状或节段性,可能代表伴有与戈谢细胞聚集相关的缺血改变的纤维性间隔。肾脏和腹部淋巴结未见异常。

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