肝豆状核变性21例患者临床分析  

Clinical analysis of 21 patients with Wilson's disease

作　　者：刘淑媛[1] 袁娟[1] 张帮杰[1] 

机构地区：[1]新乡医学院第三附属医院肝病科,河南新乡453003

出　　处：《中国民康医学》2014年第3期24-26,共3页Medical Journal of Chinese People’s Health

摘　　要：目的:探索肝豆状核变性的临床特点,提高诊断水平。方法:对21例患者肝豆状核变性的发病情况、临床表现、辅助检查及诊疗过程进行回顾性分析。结果:临床表现多种多样,肝型患者11例、神经型患者3例、混合型患者7例;K-F环阳性患者19例,占90.48%,有神经系统表现患者均阳性;血清铜、血浆铜蓝蛋白降低患者分别占80.95%和85.71%;尿铜增加患者占85.71%;误诊患者13例,误诊率61.90%。多数误诊为慢性肝病及神经系统疾病,1例患者误诊为肾炎。结论:本病误诊率高,不明原因慢性肝病、神经系统疾病及肾脏疾病通过K-F环检查、血铜、血浆铜蓝蛋白和尿铜检测对诊断有重要价值。ObjectiveTo study clinical features of Wilson＇s disease so as to elevate diagnostic level. Methods：Retrospectiveanalysis was performed on 21 patients suffering from Wilson＇s disease, including their pathogenesis conditions, clinical manifestations,adjutant examinations and diagnosis-treatment process. Results： The clinical manifestations were various and divided into hepatic type（11 cases）, nervous type （3 cases） and mixed type （7 cases）. 19 cases showed the positive K-F ring （90. 48%）, and the K-F ringwas positive for the patients who had the positive nervous system manifestations. The cases with decreased blood copper and ceruloplas-min, and increased urine copper accounted for 80. 95% （17 cases）, 85. 71% （18 cases） and 85. 71% （18 cases）, respectively.The misdiagnosis rate was up to 61. 90% （13 cases）. The most cases were misdiagnosed as chronic hepatic disease and nervous systemdiseases, and one was misdiagnosed as nephritis. Conclusions： The misdiagnosis rate of Wilson＇s disease is high. Therefore, the K-Fring examination and the detection of blood copper, ceruloplasmin and urinary copper are valuable for the diagnosis of Wilson disease.

关 键 词：肝豆状核变性 K-F环检查 血浆铜蓝蛋白 

分 类 号：R596.1[医药卫生—内科学]