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UDP-葡萄糖醛酸基转移酶缺乏和肝胆转运缺陷的黄疸大鼠的辅助性肝移植

Auxiliary liver transplantation in jaundiced rats with UDP-glucuronyltransferase deficiency and defective hepatobiliary transport.

作者信息

Jansen P L, Hess F, Peters W H, Koenders E, Jerusalem C, Corstens F H

机构信息

Division of Gastrointestinal and Liver Diseases, Academic Medical Center, Amsterdam, The Netherlands.

出版信息

J Hepatol. 1989 Mar;8(2):192-200. doi: 10.1016/0168-8278(89)90007-x.

DOI:10.1016/0168-8278(89)90007-x
PMID:2497171
Abstract

In this study auxiliary liver transplantation (ALT) has been tested as a means of correcting the UDP-glucuronyltransferase deficiency in Gunn rats and the UDP-glucuronyltransferase deficiency and impaired hepatobiliary bilirubin transport in double mutant rats. In both groups serum bilirubin normalized and remained low until the end of the study at 12 weeks after transplantation in 4 out of 6 rats. Excretion of 99mTc-HIDA in non-transplanted double mutants was considerably slower than in Gunn rats (kel 0.9 x 10(-3) versus 4.3 x 10(-3) s-1). HIDA excretion by transplants in double mutants and Gunn rats was about equal (kel 1.6 x 10(-3) and 1.1 x 10(-3) s-1). Experiments with bile duct-cannulated transplants showed that in double mutants bile flow, bile acid and bilirubin excretion was 2-4 times higher than in Gunn rats. This study shows that auxiliary liver transplants can conjugate and excrete bilirubin when one of these or both functions are lacking in the recipient's liver.

摘要

在本研究中,辅助性肝移植(ALT)已被作为一种纠正Gunn大鼠中尿苷二磷酸葡萄糖醛酸基转移酶缺乏以及双突变大鼠中尿苷二磷酸葡萄糖醛酸基转移酶缺乏和肝胆胆红素转运受损的方法进行了测试。在两组中,6只大鼠中有4只在移植后12周的研究结束时血清胆红素恢复正常并维持在低水平。未移植的双突变大鼠中99mTc-HIDA的排泄比Gunn大鼠慢得多(清除速率常数分别为0.9×10⁻³和4.3×10⁻³ s⁻¹)。双突变大鼠和Gunn大鼠中移植肝脏的HIDA排泄大致相等(清除速率常数分别为1.6×10⁻³和1.1×10⁻³ s⁻¹)。对胆管插管的移植肝脏进行的实验表明,双突变大鼠的胆汁流量、胆汁酸和胆红素排泄比Gunn大鼠高2至4倍。这项研究表明,当受体肝脏缺乏这些功能中的一种或两种时,辅助性肝移植可以结合并排泄胆红素。

相似文献

1
Auxiliary liver transplantation in jaundiced rats with UDP-glucuronyltransferase deficiency and defective hepatobiliary transport.UDP-葡萄糖醛酸基转移酶缺乏和肝胆转运缺陷的黄疸大鼠的辅助性肝移植
J Hepatol. 1989 Mar;8(2):192-200. doi: 10.1016/0168-8278(89)90007-x.
2
Hepatobiliary excretion of organic anions in double-mutant rats with a combination of defective canalicular transport and uridine 5'-diphosphate-glucuronyltransferase deficiency.具有小管转运缺陷和尿苷5'-二磷酸葡萄糖醛酸基转移酶缺乏症的双突变大鼠中有机阴离子的肝胆排泄
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Treatment of enzyme deficiency by hepatocyte transplantation in rats.大鼠肝细胞移植治疗酶缺乏症
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Prenatal diagnosis of bilirubin-UDP-glucuronosyltransferase deficiency in rats by genomic DNA analysis.
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J Clin Invest. 1978 Jul;62(1):191-6. doi: 10.1172/JCI109105.
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Isolation of an activator of bilirubin glucuronyltransferase from normal and jaundiced Gunn rats.从正常和黄疸型冈恩大鼠中分离胆红素葡萄糖醛酸转移酶激活剂。
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Supplement of liver enzyme by intestinal and kidney transplants in congenitally enzyme-deficient rat.先天性酶缺乏大鼠肠和肾移植对肝酶的补充作用
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Transplantation of Gunn rats with autologous fibroblasts expressing bilirubin UDP-glucuronosyltransferase: correction of genetic deficiency and tumor formation.将表达胆红素UDP-葡萄糖醛酸基转移酶的自体成纤维细胞移植到冈恩大鼠体内:纠正基因缺陷与肿瘤形成
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Intrahepatic hepatocyte transplantation following subtotal hepatectomy in the recipient: a possible model in the treatment of hepatic enzyme deficiency.受体肝大部切除术后肝内肝细胞移植:一种治疗肝酶缺乏症的可能模型。
J Pediatr Surg. 1992 Mar;27(3):312-5; discussion 315-6. doi: 10.1016/0022-3468(92)90853-y.

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