DuBois R S, Rodgerson D O, Martineau G, Shroter G, Giles G, Lilly J, Halgrimson C G, Starzl T E, Sternlieb I, Scheinberg I H
Lancet. 1971 Mar 13;1(7698):505-8. doi: 10.1016/s0140-6736(71)91121-4.
An 11-year-old boy with terminal hepatic failure due to Wilson’s disease was treated 18 months ago with orthotopic liver transplantation. Postoperatively, there has been evidence of clearance of body copper stores but without accumulation of copper in biopsy specimens of the transplanted liver after 6 and 17 months. Further follow-up will be necessary before deciding whether the disorder has been cured by liver replacement and in turn whether this constitutes proof that Wilson’s disease is an inborn error of hepatic metabolism. The observations so far are consistent with these conclusions.
一名因威尔逊病导致终末期肝衰竭的11岁男孩于18个月前接受了原位肝移植治疗。术后，有证据表明体内铜储备已清除，但在术后6个月和17个月时，移植肝活检标本中未出现铜蓄积。在确定该疾病是否已通过肝脏置换治愈，以及这是否反过来证明威尔逊病是一种先天性肝脏代谢紊乱之前，还需要进一步随访。目前的观察结果与这些结论一致。
