A comparative study of biliary trace elements and clinical phenotypes in Wilson disease

ＡｃｏｍｐａｒａｔｉｖｅｓｔｕｄｙｏｆｂｉｌｉａｒｙｔｒａｃｅｅｌｅｍｅｎｔｓａｎｄｃｌｉｎｉｃａｌｐｈｅｎｏｔｙｐｅｓｉｎＷｉｌｓｏｎｄｉｓｅａｓｅＲＥＮＭｉｎｇＳｈａｎ１，ＦＡＮＹｕＸｉｎ２，ＹＡＮＧＲｅｎＭｉｎ１，ＨＡＮＹｏｎｇＺｈｕ...

IM To further explore the etiological mechanism of Wilson disease (WD), by comparing the changes of biliary trace elements and its clinical phenotype.METHODS Wilson disease patients with different types and conditions (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 copper content and copper/zinc ratio of biliary juice were evidently lower than those of nonWD patients with chronic liver damage and healthy controls (F=1476, 254; 1492, 262; P<001, respectively), while biliary zinc level had no significant difference from two nonWD control groups (P>005). There were significant differences in biliary copper excretion among patients with different types and conditions (F=375, P<005; F=620, P<001).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 closely related with the severity of hepatic injury and illness.