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。
Objective To determine the clinical manifestation of children with hepatolentieular 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 liver dysfunction and hemolytic anemia as the first signs or symptoms. Only 5 cases （7. 46% ） had specific symptoms of nervous system involvement. Misdiagnosis for hepatolentieular degeneration included viral hepatitis, liver cirrhosis, nephritis and hematological diseases. Conclusion Children with hepatolentieular degeneration frequently have various symptoms and signs. Unrecognized liver dysfunction, liver cirrhosis, hepatic function failure and/or hemolytic anemia may therefore indicate hepatolenticular degeneration. Further laboratory test are needed to make a correct diagnosis and prevent misdiagnosis.
出处
《广州医药》
2008年第6期20-22,共3页
Guangzhou Medical Journal
关键词
肝豆状核变性
儿童
诊断
临床表现
Hepatolenticular degeneration
Children
Diagnosis
Clinical manifestation
