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| 肝豆状核变性23例临床分析 | |
| 引用本文： | 王冬青,蔺慕会,龚涛. 肝豆状核变性23例临床分析[J]. 中国神经免疫学和神经病学杂志, 2011, 18(2). DOI: 10.3969/j.issn.1006-2963.2011.02.006 |
| 作者姓名： | 王冬青 蔺慕会 龚涛 |
| 作者单位： | 1. 辽宁省盘锦市第二人民医院神经内科,124000 2. 辽宁省人民医院神经内科,110016 3. 卫生部北京医院神经内科,100730 |
| 摘 要： | 目的 总结肝豆状核变性的临床特点,以减少误诊、漏诊.方法 回顾性分析23例肝豆状核变性患者的临床表现及诊治过程.结果 平均发病年龄21.5岁,首发症状以神经系统和肝损害症状为主,分别占69.6%(16/23)及26.1%(6/23),其中神经系统症状以肢体震颤、精神异常多见;18例患者临床分型为混合型,占78.3%(18/23),5例为脑型;所有患者均出现角膜色素环(K-F环);所有患者进行了铜代谢的实验室检查,血浆铜蓝蛋白水平降低及24 h尿铜增高常见.青霉胺与硫酸锌联合治疗对78.3%(18/23)的患者有效.结论 肝豆状核变性青少年多发,以神经系统和肝损害症状为主要表现,K-F环阳性率高.青霉胺与硫酸锌联合治疗对大部分肝豆状核变性患者有效. |
| 关 键 词： | 肝豆状核变性 临床表现 治疗 |
The clinical analysis of 23 patients with hepatolenticular degeneration |
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| WANG Dong-qing,LIN Mu-hui,GONG Tao. The clinical analysis of 23 patients with hepatolenticular degeneration[J]. Chinese Journal of Neuroimmunology and Neurology, 2011, 18(2). DOI: 10.3969/j.issn.1006-2963.2011.02.006 | |
| Authors: | WANG Dong-qing LIN Mu-hui GONG Tao |
| Affiliation: | WANG Dong-qing,LIN Mu-hui,GONG Tao.Department of Neurology,The Second People Hospital of Panjin,Panjin Liaoning 124000,China |
| Abstract: | Objective To summarize the clinical characteristics of hepatolenticular degeneration(Wilson disease,WD) so that to decrease the misdiagnosis and missed diagnosis.Methods The clinical manifestations,diagnosis and treatment process of 23 patients with WD were analyzed retrospectively.Results The mean occurrence age was 21.5.The first-ever symptoms were mainly nervous system and liver lesion,69.6%(16/23) and 26.1%(6/23)respectively.Limb tremor and psychiatric disorder were mainly found in nervous system lesion... |
| Keywords: | hepatolenticular degeneration(Wilson disease) clinical manifestations treatment |
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