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肝豆状核变性的脑部病变主要在( )

发布日期:2020-08-20

肝豆状核变性的脑部病变主要在( )
A

大脑皮质

B

基底神经节

C

小脑半球

D

丘脑黑质

E

脑干网状系统

试题解析

肝豆状核变性

肝豆状核变性(hepatolenticular degeneration,HLD)由Wilson在1912年首先描述,故又称为Wilson病(Wilson Disease,WD)。是一种常染色体隐性遗传的铜代谢障碍性疾病,以铜代谢障碍引起的肝硬化、基底节损害为主的脑变性疾病为特点,对肝豆状核变性发病机制的认识已深入到分子水平。WD的世界范围发病率为1/30 000~1/100 000,致病基因携带者约为1/90。本病在中国较多见。WD好发于青少年,男性比女性稍多,如不恰当治疗将会致残甚至死亡。WD也是少数几种可治的神经遗传病之一,关键是早发现、早诊断、早治疗。2018年5月11日,国家卫生健康委员会等5部门联合制定了《第一批罕见病目录》,肝豆状核变性被收录其中。

外文名
hepatolenticular degeneration,HLD
传染性
无传染性
所属科室
内科 - 神经内科
常见发病部位
大脑
西医学名
肝豆状核变性

病变

《BINGBIAN病变》女生版由鞠文娴演唱,Jurrivh作曲,苏州文弘公司出品。

中文名
病变
专辑语言
普通话
专辑时长
0时04分07秒
音乐风格
流行
发行日期
2018年1月27日
制作人
Jurrivh
外文名
lesion
专辑歌手
鞠文娴
曲目数量
1 首
唱片公司
苏州文弘
发行地区
大陆

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此试题出现在

医学高级内科

小儿内科学(医学高级)

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