摘要
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，４４±０４ｖｓ４１７±２０，４２０±２６）和铜／锌比值（０１３±００２ｖｓ１５４±０２７，１５６±０２４）显著低于有慢性肝损害的非ＷＤ患者及健康自愿者（Ｐ＜００１），而胆汁锌含量无明显差异（Ｐ＞００５）．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＜００５；Ｐ＜００１）．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 disease, the biliary copper content ( μmol/L , 4 4±0 4 vs 41 7±2 0, 42 0±2 6) and copper/zinc ratio (0 13±0 02 vs 1 54±0 27, 1 56±0 24) were evidently lower than those of non WD patients with chronic liver damage and healthy controls ( P <0 01, respectively), while biliary zinc level had no significant difference compared with two non WD control groups ( P >0 05). Significant differences existed in biliary copper excretion among different clinical phenotypes and conditions of WD ( P <0 05, P <0 01, respectively). CONCLUSION Copper excretion by liver and biliary system decreases obviously, which plays a key role in the copper retention of WD, and the biliary copper retention is consistent well with the severity of hepatic injury and illness.
关键词
肝豆状核变性
病因学
分光光度法
hepatolenticular degeneration
bile/chemistry
copper/analysis
zinc/analysis
liver diseases/etiology
spectrophotometry, atomic absorption
