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儿童肝豆状核变性67例临床分析
引用本文:陈惠芹,张涤华,蔡耘,张萍萍,王清文,何政贤.儿童肝豆状核变性67例临床分析[J].广州医药,2008,39(6).
作者姓名:陈惠芹  张涤华  蔡耘  张萍萍  王清文  何政贤
作者单位:1. 中山大学附属第三医院儿科,广州,510630
2. 中山大学附属第一医院内科,广州,510080
摘    要:目的 分析儿童肝豆状核变性的常见临床表现,以减少误诊.方法 对67例儿童肝豆状核变性患者的性别、年龄、首发症状及误诊病种进行回顾性分析.结果 本组儿童肝豆状核变性患者以肝损害首发者最为常见,占70.15%,以肝损害同时伴有溶血性贫血首发者次之,占22.39%,以神经系统症状首发者相对少见,占7.46%.误诊为病毒性肝炎、肝硬化者居多,少数误诊为肾炎、血液病.结论 儿童肝豆状核变性首发症状各异,临床表现多样,临床医生应加深对肝豆状核变性的认识,遇到不明原因的转氨酶升高、青少年期肝硬化、急性肝功能衰竭、溶血性贫血合并肝损害,应考虑肝豆状核变性的可能 ,及早明确诊断.

关 键 词:肝豆状核变性  儿童  诊断  临床表现

A retrospective analysis of 67 cases of hepatolenticular degeneration in children
Chen Huiqin,Zhang Dihua,Cai Yun,et al..A retrospective analysis of 67 cases of hepatolenticular degeneration in children[J].Guangzhou Medical Journal,2008,39(6).
Authors:Chen Huiqin  Zhang Dihua  Cai Yun  
Affiliation:Chen Huiqin1,Zhang Dihua2,Cai Yun1,et al.1 Department of Pediatrics,the Third Affiliated Hospital,Sun Yat-sen University,Guangzhou 510630,China.2 Department of Internal Medicine,the First Affiliated Hospital,Guangzhou 510080,China
Abstract:Objective To determine the clinical manifestation of children with hepatolenticular degeneration and prevent misdiagnosis.Methods A retrospective study of 67 cases of children with hepatolenticular degeneration was conducted.Data collected included gender,age,clinical manifestation,and causes for misdiagnosis. Results Of 67 children diagnosed with hepatolenticular degeneration,47 cases(70.15%) had liver dysfunction which was the most common and chief clinical manifestation.15 cases(22.39%) presented with li...
Keywords:Hepatolenticular degeneration  Children  Diagnosis  Clinical manifestation  
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