الفهرس 
Acknowledgement
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Abstract
Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) represent major indications for liver transplantation (LT); In general, LT in Cholestatic liver diseases CD patients is indicated when liver failure occurs with complications similar to those for end-stage liver disease caused by other etiologies. An unacceptable quality of life because of severe, treatment-resistant pruritus or severe hepatic encephalopathy may also merit consideration for transplantation. PBC is a chronic autoimmune disease that mainly targets biliary epithelial cells. Its complex etiology involves an interaction between genetic susceptibility and environmental factors .One-third of PBC patients may require LT, and PSC is a chronic, immune-mediated cholestatic liver disease that mainly affects intra and extrahepatic bile ducts, causing progressive inflammation and obliterative fibrosis, promoting multiple strictures PBC and PSC recur in many recipients, and recurrence may be more aggressive than the original diseaseto analyse patients transplanted for PSC and PBC, with a long period of follow-up, in order to evaluate the incidence of complications and disease recurrence, to assess the impact on survival after LT, and to evaluate various regimens of immunosuppression therapy, in comparison to those patients transplanted for other most common causes of liver transplantation including HBV, HCV and ALD but excluding HCC. The present study included liver transplanted patients in three groups; (group I); enrolled 61 patients who were transplanted for Primary sclerosing cholangitis (PSC), (group II); enrolled 50 patients who were transplanted for primary biliary cholangitis (PBC) and (group III) included 73 patients who were transplanted for other causes with exclusion of HCC. Regarding gender there was statistically significant difference in between groups ; in PBC group; females represented the majority (88.5 %), in PSC group males was (66%), in the group III males was the major component (87.7%) For Age at the time of transplantation, the mean ± SD age in PBC group was 52.54 ± 8.91, in PSC was 43.20 ± 14.91 and in group III was 53.10 ± 8.64, with highly statistically significant difference between PBC and PSC and between PSC and group III , but no statistically significant difference was found between PBC and group IIIIntractable symptoms were statistically significant higher in PSC group (40%) than PBC group (14.8%).Regarding biliary complication; biliary leak was found in 9.8%,8% and 12.3% for PBC, PSC and group III respectively, with no statistically significant difference between groups; biliary stricture: was found in 3.3% in PBC,6% in PSC and 8.2% in group III, with no statistically significant difference , and Biliary stenting: was found in 3.3 % , 8% and 6.8% for PBC, PSC and group III respectively, with no statistically significant difference biliary leak was found in 9.8%,8% and 12.3% for PBC, PSC and group III respectively, biliary stricture: was found in 3.3% in PBC,6% in PSC and 8.2% in group III, and Biliary stenting: was found in 3.3 % , 8% and 6.8% for PBC, PSC and group III respectively, with no statistically significant difference. The percentage of patients who received post-transplant Tac+AZA immunosuppression was 67.2% in the PBC group, 70.0% in the PSC group, and 86.3% in the third group. In cases of PBC, there was no difference between the two regimens regarding recurrence at one year (20.0% for Cyc+AZA and 19.5% for Tac+AZA respectively. In PSC group, the one-year recurrence rates were 13.3% with the Cyc+AZA regimen and 14.3% with Tac+AZA regimen. That was statistically insignificant. The outcome for participants overall survival was in (98.4%, 96% and 97.3%) of PBC, PSC and group III respectively with no statistically significant difference according to present study.Regarding gender there was statistically significant difference between groups; in PBC group; females represented the majority (88.5 %), in PSC group males was (66%), in the group III males was the major component (87.7%).For Age at the time of transplantation, the mean ± SD age in PBC group was 52.54 ± 8.91, in PSC was 43.20 ± 14.91 and in group III was 53.10 ± 8.64, with highly statistically significant difference between PBC and PSC and between PSC and group III , but no statistically significant difference was found between PBC and group III. Intractable symptoms were statistically significantly higher in the PSC group (40%) than PBC group (14.8%).Regarding biliary complication, there was no statistically significant difference between the outcomes, incidence of biliary leaks and strictures and the need for biliary stenting in the three groups. The percentage of patients who received post-transplant Tacrolimus as the main CNI immunosuppression was higher in all groups. There was no statistical difference between the two regimens (tacrolimus-based or cyclosporine-based) regarding recurrence at one year in all groups. Overall survival at one year was not statistically significant between the three groups. The use of a specific immunosuppression protocol in the postoperative treatment of liver transplant patient in the three groups did not show any statistical significance in terms of effects on postoperative complications (including biliary complications), hospital or ICU stay or disease recurrence at one year. Patients with PBC tend to have more total hospital stay (ICU+ ward) than the other two groups. Recurrence rates were 19.7% for the PBC group and 14% for the PSC group, with no statistically significant difference regarding complication occurrence among different groups. Liver transplantation in cases of primary biliary diseases (PBC and PSC) is an excellent treatment option with excellent one-year survival rate and a complication rate similar to other etiologies. Recurrence of PBC and PSC is rare at one year and rarely requires re-transplantation. Further research is required in search for new options to treat patients with PSC and PBC with intractable itching who did not reach the threshold MELD score for listing to avoid liver transplantation with all its intra and post-operative complications. There is no need to specify a fixed immunosuppression regimen in PBC and PSC patients after liver transplantation as both protocols (tacrolimus-based and cyclosporine-based) appear to be safe with low complication rates and post-transplant recurrence. There might be a need to further replicate our study with higher number of patients and longer periods of follow up (5-10 years) to investigate the long-term outcomes in those patients. It is important to re-emphasise that our study was conducted on deceased-donor liver transplants (DDLT), further research may be required on living-related liver transplantation (LDLT) to discover if there is any differences especially in the rate of biliary complications and the immunosuppressives used