Whitington P F, Emond J C, Heffron T, Thistlethwaite J R
Department of Pediatrics, University of Chicago Pritzker School of Medicine, Wyler Children's Hospital, IL.
Lancet. 1993 Sep 25;342(8874):779-80. doi: 10.1016/0140-6736(93)91543-u.
Some diseases that result from inborn errors of critical metabolic or synthetic processes mainly involving the liver do not cause structural liver damage. These disorders can be treated by the addition of liver tissue (auxiliary liver transplantation) rather than liver replacement. We report correction of the metabolic error in a 13-year-old girl with Crigler-Najjar syndrome type 1 by auxiliary (left lateral segment) transplantation. The first graft failed and was replaced successfully. The second graft shows features of chronic rejection, but at 2 years postoperatively bilirubin conjugating ability has not been impaired. Another graft may become necessary in due course.
一些主要累及肝脏的关键代谢或合成过程先天性缺陷所导致的疾病，并不会引起肝脏结构损伤。这些病症可通过添加肝组织（辅助性肝移植）而非进行肝脏置换来治疗。我们报告了通过辅助性（左外侧叶）肝移植纠正一名患有1型克里格勒 - 纳贾尔综合征的13岁女孩代谢错误的情况。首次移植失败后成功进行了再次移植。第二次移植显示出慢性排斥反应的特征，但术后2年胆红素结合能力并未受损。在适当的时候可能需要进行另一次移植。
