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一名出现髋部和腹部疼痛的患者患有戈谢病。

Gaucher's disease in a patient presenting with hip and abdominal pain.

作者信息

Resorlu Mustafa, Aylanc Nilufer, Karatag Ozan, Toprak Canan Akgun

机构信息

Department of Radiology, Faculty of Medicine, Canakkale Onsekiz Mart University, Canakkale, Turkey.

出版信息

Rev Assoc Med Bras (1992). 2017 Dec;63(12):1025-1027. doi: 10.1590/1806-9282.63.12.1025.

Abstract

Gaucher's disease is characterized by glucocerebroside accumulation in the cells of the reticuloendothelial system. There are three subtypes. The most common is type 1, known as the non-neuropathic form. Pancytopenia, hepatosplenomegaly and bone lesions occur as a result of glucocerebroside accumulation in the liver, lung, spleen and bone marrow in these patients. Findings associated with liver, spleen or bone involvement may be seen at radiological analysis. Improvement in extraskeletal system findings is seen with enzyme replacement therapy. Support therapy is added in patients developing infection, anemia or pain. We describe a case of hepatosplenomegaly, splenic infarction, splenic nodules and femur fracture determined at radiological imaging in a patient under monitoring due to Gaucher's disease.

摘要

戈谢病的特征是网状内皮系统细胞中葡糖脑苷脂蓄积。它有三种亚型。最常见的是1型,即非神经病变型。这些患者由于葡糖脑苷脂在肝脏、肺、脾脏和骨髓中蓄积,会出现全血细胞减少、肝脾肿大和骨病变。在放射学分析中可见与肝脏、脾脏或骨骼受累相关的表现。酶替代疗法可使骨骼外系统表现得到改善。对于发生感染、贫血或疼痛的患者,需增加支持治疗。我们描述了一例因戈谢病接受监测的患者,其在放射学成像中发现有肝脾肿大、脾梗死、脾结节和股骨骨折。

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