SMALL BOWEL TRANSPLANT TO SUPPLEMENT THE ENZYME FOR HYPERBILIRUBINEMIA IN GUNN RATS.

我们观察了遗传性胆红素UDP-GT缺陷型Gunn大鼠的全肠移植和部分肠移植的效果，以期建立一种新的补充缺陷酶的方法。实验分组如下：1：正常WS大鼠(RT1^K)，2：未治疗的Gunn大鼠(JJ，RT1^K)，3：全小肠移植(TSBTx)，4：20 cm空肠移植(J20SBTx)，5：20 cm回肠移植(I20SBTx)，6：10 cm空肠移植(J10SBTx)。血清总胆红素(TB)水平为0.27。正常WS0.02 mg/dl，Gunn大鼠7.36+-Gt；0.68 mg/dl。所有同种异体移植组的TB水平均显著低于Gunn大鼠，但未降至正常WS大鼠水平。TSBTx的TB水平在同种异体移植组中最低，J10SBTx移植后1个月与移植后1个月相比有显著差异。移植后4个月胆汁胆红素分析显示，随着SBTx的增加，结合胆红素显著升高，表明受体的胆红素结合能力提高。胆红素结合的效果似乎与移植物的长度有关，而与移植物的来源无关。结果表明，部分SBTx和总SBTx均能显著降低Gunn大鼠血清TB水平，升高胆汁结合胆红素水平。

We observed the effect of total and partial bowel transplantation in hereditary bilirubin UDP-GT deficient Gunn rats, as an Crigler-Najjar syndrome type 1, in an attempt to establish a new method for supplying the deficient enzyme.The experimental groups were as follows ; 1 : normal WS rats (RT1^K), 2 : untreated Gunn rats (jj, RT1^K), 3 : total small bowel transplant (TSBTx), 4 : 20cm jejunum transplant (J20SBTx), 5 : 20cm ileum transplant (I20SBTx), 6 : 10cm jejunum transplant (J10SBTx).Serum total bilirubin (TB) levels were 0.27<plus-minus>0.02mg/dl in normal WS and 7.36<plus-minus>0.68mg/dl in untreated Gunn rats. All groups with isografting showed significantly lower TB levels than Gunn rat, but they did not decrease to the level of normal WS rats. TB level in TSBTx was lowest among isografted groups and a significant difference was noted from 1 month posttransplant in J 10SBTx. Bile bilirubin analysis 4 months posttransplant showed conjugated bilirubin increased significantly follwing each kind of SBTx, suggesting improved bilirubin conjugation ability of recipients. The effectiveness of bilirubin conjugation seems correlated to the length of grafts rather than origin of graThese results demonstrated that partial SBTx as well as total SBTx significantly decreased serum TB levels and increased bile conjugated bilirubin in Gunn rats.