威尔逊病的原位肝移植
Orthotopic liver transplantation for Wilson's disease.
作者信息
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
出版信息
Lancet. 1971 Mar 13;1(7698):505-8. doi: 10.1016/s0140-6736(71)91121-4.
Abstract
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.
摘要
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。
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本文引用的文献
1
DETECTION OF THE HETEROZYGOUS CARRIER OF THE WILSON'S DISEASE GENE.威尔逊病基因杂合携带者的检测
J Clin Invest. 1961 Apr;40(4):707-15. doi: 10.1172/JCI104304.
2
IMMUNOSUPPRESSION AFTER EXPERIMENTAL AND CLINICAL HOMOTRANSPLANTATION OF THE LIVER.实验性及临床肝脏同种移植后的免疫抑制
Ann Surg. 1964 Sep;160(3):411-39. doi: 10.1097/00000658-196409000-00007.
3
PENICILLAMINE THERAPY FOR HEPATOLENTICULAR DEGENERATION.青霉胺治疗肝豆状核变性
JAMA. 1964 Sep 7;189:748-54. doi: 10.1001/jama.1964.03070100042008.
4
FAMILIAL HEPATIC COPPER STORAGE DISEASE: A VARIANT OF WILSON'S DISEASE.家族性肝铜贮积病：威尔逊病的一种变体
Arch Dis Child. 1964 Feb;39(203):14-7. doi: 10.1136/adc.39.203.14.
5
RENAL LESIONS IN WILSON'S DISEASE.威尔逊氏病中的肾脏病变
Lancet. 1964 Apr 18;1(7338):843-5. doi: 10.1016/s0140-6736(64)91573-9.
6
Wilson's disease. The presenting symptoms.威尔逊氏病。首发症状。
Arch Dis Child. 1962 Jun;37(193):253-6. doi: 10.1136/adc.37.193.253.
7
Treatment of Wilson's disease with penicillamine.用青霉胺治疗威尔逊氏病。
Lancet. 1960 Jan 23;1(7117):188-92. doi: 10.1016/s0140-6736(60)90109-4.
8
Renal function in Wilson's disease.威尔逊病中的肾功能。
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