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以肝损害为首发症状的肝豆状核变性超声声像分析
引用本文:孙德胜,鲁树坤,言伟强,王丹军,王双双,刘晓平.以肝损害为首发症状的肝豆状核变性超声声像分析[J].罕少疾病杂志,2005,12(4):12-14.
作者姓名:孙德胜  鲁树坤  言伟强  王丹军  王双双  刘晓平
作者单位:北京大学深圳医院,广东,深圳,518036;北京大学深圳医院,广东,深圳,518036;北京大学深圳医院,广东,深圳,518036;北京大学深圳医院,广东,深圳,518036;北京大学深圳医院,广东,深圳,518036;北京大学深圳医院,广东,深圳,518036
摘    要:目的 分析以肝脏损害为首发症状的肝豆状核变性的声像图特点。方法 回顾性分析我院收治的27例肝豆状核变性患者的超声声像资料,并复习相关文献。结果27例中男12例,女15例,起病年龄5-53岁,平均18.5岁。肝脏声像图主要表现为光点粗密、增强。其中单纯增强型8例,类似脂肪肝声像:结节硬化型19例,表现为树枝状、网格状回声增强及结节样改变,伴有肝内管系结构紊乱:部分患者伴有脾脏肿大、胆囊结石、慢性胆囊炎等。结论 肝豆状核变性肝脏声像图改变明显,超声检查可为该病诊断提供线索。

关 键 词:肝豆状核变性  超声检查
文章编号:1009-3257(2005)04-0012-03
收稿时间:2005-04-18
修稿时间:2005年4月18日

Sonographic study on hepatolenticular degeneration with early liver damage
Sun DeSheng;Lu ShuKun;Yan WeiJiang;Wang DanJun;Wang ShuangShuang;Liu XiaoPing.Sonographic study on hepatolenticular degeneration with early liver damage[J].Journal of Rare and Uncommon Diseases,2005,12(4):12-14.
Authors:Sun DeSheng;Lu ShuKun;Yan WeiJiang;Wang DanJun;Wang ShuangShuang;Liu XiaoPing
Abstract:Objective To analyze the sonographic features of hepatolenticular degeneration (HLD) with liver damage. Methods The ultrasonic findings of 27 HLD patients were studied retrospectively, and some related medical literature was reviewed. Results Of 27 patients, 12 were male and 15 were female, whose age ranged from 5 to 53 years,and the average was 18.5 years. Ultrasonography showed that the parenchyma echo pattern was of increased echogenicity. The echo texture was coarse, diffusely enhanced. There were 8 cases of pure enhanced type without focal lesions, and 19 cases of cirrhosis nodus like type with dysplastic alterations, including branche or net like enhance. Some patients were companied by spleen tumefaction, cholelithiasis and chronic cholecystitis. Conclusion The HLD patients are of identified ultrasonic features. Ultrasonography play an important role in diagnosis of HLD.
Keywords:hepatolenticular degeneratiom ultrasonography
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