Gaucher disease in the liver on hepatocyte specific contrast agent enhanced MR imaging

被引：0
作者：
Rama S. Ayyala
Lisa A. Teot
Jeanette M. Perez Rossello
机构：
[1] Morgan Stanley Children’s Hospital,Department of Radiology, Columbia University Medical Center
[2] Boston Children’s Hospital,Department of Pathology, Harvard Medical School
[3] Boston Children’s Hospital,Department of Radiology, Harvard Medical School
来源：
Pediatric Radiology | 2017年 / 47卷
关键词：
Child; Gaucher disease; Hepatocyte specific contrast agents; Liver; Magnetic resonance imaging;
D O I：
暂无
中图分类号：
学科分类号：
摘要：
Gaucher disease is a hereditary lipid storage disorder that affects the enzyme beta glucocerebrosidase, causing accumulation of glucocerebroside in macrophages of the reticuloendothelial system. Accumulation can occur in the liver and spleen, manifesting as hepatosplenomegaly, as well as within the bone marrow. Hepatic involvement is usually diffuse but can occasionally manifest as focal liver lesions. We present a case of a 2-year-old boy with Gaucher disease and an infiltrating liver lesion detected on imaging, which was pathologically shown to be focal changes related to the disease. Imaging characteristics of this lesion using hepatocyte specific contrast agent enhanced MRI, which have not been previously discussed in the literature, are described.
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页码：484 / 487
页数：3
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