Goss J A, Stribling R, Martin P
Dumont-University of California Los Angeles Transplant Center, Division of Liver and Pancreas Transplantation, Los Angeles, California 90095-7054, USA.
Clin Liver Dis. 1998 Feb;2(1):187-210. doi: 10.1016/s1089-3261(05)70371-8.
Liver replacement provides an effective method of replacing a failing liver, and corrects the underlying defect in many metabolic conditions. Results of liver transplantation for metabolic diseases have been encouraging, with the exception of hereditary hemochromatosis, in which infectious and for which cardiac complications appear to increase posttransplant mortality. An improved understanding of the underlying genetic and molecular defect will lead to advances in medical therapy and perhaps will decrease the need for liver replacement. The prospects of gene therapy are being pursued for many metabolic disorders, however until this research leads to direct clinical application, liver transplantation remains the only effective option for many patients with metabolic liver disease.
肝脏置换提供了一种替换衰竭肝脏的有效方法,并纠正了许多代谢性疾病的潜在缺陷。除遗传性血色素沉着症外,代谢性疾病的肝移植结果令人鼓舞,在遗传性血色素沉着症中,感染以及心脏并发症似乎会增加移植后的死亡率。对潜在遗传和分子缺陷的进一步了解将推动医学治疗的进展,或许还会减少肝脏置换的需求。目前正在对许多代谢紊乱疾病进行基因治疗的探索,然而,在这项研究导致直接临床应用之前,肝移植仍然是许多代谢性肝病患者的唯一有效选择。