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

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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.