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机构地区:[1]山东省威海市立医院眼科,山东威海264200 [2]中山大学附属第一医院神经科,广东广州510080
出 处:《中国现代医学杂志》2013年第3期44-47,共4页China Journal of Modern Medicine
基 金:国家自然科学基金(No:30671154)
摘 要:目的探讨角膜K-F环对肝豆状核变性(Wilson病)的诊断价值。方法对200例确诊Wilson病患者的症状、体征及主要辅助检查资料进行分析。结果本组患者最常见的临床表现为震颤(42.50%);以脑型患者最多,122例(61.00%);铜蓝蛋白检查194例,均<0.15 g/L,其中178例≤0.08 g/L;角膜K-F环检出率为85.5%;脑型患者中K-F环阳性率99.18%(121/122)。经Logistic回归分析发现年龄是角膜K-F环出现与否的独立相关因素,(r=0.938,P<0.05)。结论角膜K-F环是诊断Wilson病的金标准之一。年龄为出现角膜K-F环的独立相关因素。脑型患者角膜K-F环阳性检出率高于肝型。经治疗角膜K-F环减弱或消失,说明患者驱铜治疗有效。[ Objective] To discuss the values of Kayser-Fleischer ring in the diagnosis of Wilson Disease. [Methods] Analyzed the clinical data and accessory examination results of 200 patients diagnosed by the criteria of Wilson Disease. [Results] The prominent clinical features of Wilson Disease was tremor. CP is below 0.15 g/L in all the patients who were detected and 92.00% of which below 0.08 g/L. Kayser-Fleischer ring were found in 85.50% of all the patients and 99.18% of patients with neurological or psychiatric symptoms. Age is independent factor for ap- pearance of Kayser-Fleischer ring. [ Conclusion] Kayser-Fleiseher ring is an excellent discriminatory test for the diagnosis of Wilson Disease. The disappearance or reduction is independent of the stage of the disease as also the effectiveness of copper chelation and is not a good predictor of clinical improvement in patients.
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