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目的研究WILSON病(WD)患者和非WD对照者胆汁铜、锌含量的变化,结合临床表型进一步探讨WD铜滞留的病因机制.方法不同分型、病情各异的WD患者20例,有慢性肝损害的非WD患者22例和健康自愿者10例,均实施十二指肠引流术留取胆汁样本,采用原子吸收分光光度计检测各样本的铜、锌含量.结果WD患者的胆汁铜含量(μMOL/L,4?4±0?4VS41?7±2?0,42?0±2?6)和铜/锌比值(0?13±0?02VS1?54±0?27,1?56±0?24)显著低于有慢性肝损害的非WD患者及健康自愿者(P＜0?01),而胆汁锌含量无明显差异(P＞0?05).不同分型和病情状况的WD患者胆汁铜含量存有显著性差异(P＜0?05;P＜0?01).结论肝胆系统铜排泄显著减少是WD患者铜滞留的关键,胆汁铜滞留与WD患者肝损害的程度和病情轻重有密切关系.“,”AIM To investigate the changes of biliary Cu and Zn in Wilson disease (WD) patients and non WD and further explore the etiological mechanism of WD based on clinical phenotypes. METHODS Different types of WD patients ( n =20), non WD patients with chronic liver damage ( n =22) and healthy volunteers ( n =10) as controls were studied. Biliary samples were taken by duodenal drainage. Atom absorption spectrophotometer was used to assay the copper and zinc content of each sample. RESULTS In Wilson ...