Objective: There are several studies reporting the therapeutic effects of Berberis vulgaris on liver diseases. This study was done with the purpose of examining the effect of B. vulgaris oxymel (BO) in patients with refractory primary sclerosing cholangitis (PSC) and primary biliary cholangitis (PBC), who did not respond to current treatment. Materials and Methods: Patients with PSC or PBC who were receiving ursodeoxycholic acid (UDCA, 13-15 mg/kg/day) for at least six months, but their serum levels of alkaline phosphatase (ALP) were still 1. 5 folds higher than the normal upper limit during the last six months, were asked to participate in this quasi-experimental study. Patients were asked to take 0. 5 ml/kg/day of BO two times a day for three months along with UDCA. At the end of the study, serum levels of ALP, aspartate aminotransferase (AST), alanine aminotransferase (ALT), gamma-glutamyl transferase (GGT), total bilirubin (TB), direct bilirubin (DB), and creatinine as well as prothrombin time (PT), international normalized ratio (INR) and quality of life (QOL) based on PBC-40 questionnaire were assessed as outcomes. Results: Our results showed that BO notably attenuated the serum levels of ALP, AST, ALT, GGT, TB, and DB, as well as PT and INR and significantly improved QOL. Conclusion: For first time, we showed that additional therapy with BO has a promising effect in the treatment of refractory PSC and PBC.

Background: Traditionally, Roux-en-Y hepaticojujenostomy was the method of choice for biliary reconstruction in primary sclerosing cholangitis (PSC) in patients undergoing orthotopic liver transplantation.Objective: To compare the results of duct to duct anastomosis vs Roux-en-Y hepaticojujenostomy for biliary reconstruction in patients with PSC who underwent liver transplant in Shiraz Organ Transplant Center.Methods: We reviewed records of 69 patients with PSC who underwent liver transplant. We performed duct to duct reconstruction in those patients who had a grossly normal bile duct during hepatectomy (n=29). Roux-en-Y hepaticojujenostomy was used in the remaining 40 cases. Data collected included biliary complications (leakage, stricture, stone, and cancer in the remnant bile duct), documented episodes of rejection, and morbidity.Results: The studied patients had a mean follow-up of 27 (range: 9–46) months. In duct to duct group, 2 patients presented with anastomotic stricture and 1 developed cholangiocarcinoma in distal bile duct and underwent pancreaticoduodenectomy. In Roux-en-Y group, 5 patients developed anastomotic stricture in the follow-up. The difference was not significant. The rate of documented episodes of rejection was similar in the two groups (p=0.66).Conclusion: Duct to duct reconstruction is safe and can be the method of choice for biliary reconstruction in patients with PSC. Furthermore, considering the innovations in ERCP, management of strictures in duct to duct group can be easier and more feasible in comparison to revision of Roux-en-Y hepaticojujenostomy.

primary sclerosing cholangitis is a chronic cholestatic liver disease defined by strictures of the biliary tree which could ultimately lead to liver cirrhosis and cholangiocarcinoma. Although the exact underlying etiology of this disorder is not fully understood, the pathology is believed to be caused by immune mediated mechanisms. Growing body of evidence suggests several treatment modalities mainly focusing on the inflammation aspect of this disorder. However, there is still no consensus regarding the best treatment option for these patients. Thus, the present study aimed to review the current treatment options for patients with primary sclerosing cholangitis.

Background: While Roux-en-Y hepaticojejunostomy (RYH) is the common anastomotic technique for liver transplantation (LT) in patients with primary sclerosing cholangitis (PSC), duct-to-duct (DD) reconstruction may be used if the recipient common bile duct is normal. There are conflicting observational data on the rate of success of DD reconstruction versus RYH, in PSC.Objectives: The aim of this study was to assess the safety and efficacy of DD anastomosis, compared to RYH reconstruction, among adults transplanted for PSC.Patients and Methods: All adult patients, who underwent primary LT for PSC between 1990 and 2012, were evaluated, according to type of biliary reconstruction. Recipient and graft survival, postoperative medical and surgical complications, and postoperative resource utilization rates were compared between the two groups.Results: Totally, 73 patients fulfilled the inclusion criteria. Of them, 58 had RYH and 15 had DD reconstruction. A total of 53 subjects (73%) were male, with the mean age±standard deviation at LT of 43.3±14.4 years. Rates of recipient mortality, graft failure, biliary complications, acute cellular rejection, and reoperation were similar in both groups. Postoperative cholangiography was used more frequently in patients with DD reconstruction (33.3% vs. 8.6%, P=0.026).Conclusions: In selected recipients with PSC, DD reconstruction is a safe and efficacious technique, with long-term clinical outcomes comparable to RYH.

Introduction and Aims: We noticed in our practice that the patients with ulcerative colitis (UC) who have developed primary sclerosing cholangitis (PSC) experience a milder course of colonic disease. Our objective in this study was to define whether there is any difference between UC activity and its course in patients with and without PSC. Materials and Methods: 19 patients with UC and PSC (8 male, mean age 25) were enrolled. To every patient with UC and PSC, 3 patients with UC alone (totally 57 patients, 28 male, mean age 24) matched for age at onset, duration of the disease, and extension of colonic disease were selected as the control group. We used number of hospitalizations due to activity of UC and number of short corticosteroid administrations in various years of follow-up as variables indicating course and severity of the colonic disease in this period. For comparing trends of UC activity between two groups, we used repeated measures two-way analysis of variances. Results: Mean duration of follow up in case and control groups was 12.2 ± 5.7 and 11.4 ± 4.9 years, respectively. Two groups had no significant difference in use of sulfasalazine or aminosalicylates. Number of hospitalizations and courses of steroid therapy because of UC activity decreased significantly over time (p<.000) in both groups and it was significantly higher in controls than in cases (p=.045 and .032, respectively). Conclusions: Development of PSC in patients with UC might have a positive effect on the colonic disease. Further investigation to evaluate basis of this improvement are warranted

Background: Multiple sclerosis (MS) is an autoimmune disorder of the central nervous system which damages the myelin and axon. primary biliary cholangitis (PBC) is a slow progressive liver disease with autoimmune feature in which non-purulent destructive cholangitis and interlobular bile duct destruction occur. Involvement of each of PBC and MS is thought to be related to environmental exposure in genetically susceptible persons. Case Presentation: Here, we aim to report 3 women 52, 27 and 51 years old with MS and PBC. Conclusion: Although MS seems to have an association with some autoimmune gastrointestinal disorders such as ulcerative colitis, the concurrence of MS and PBC has been rarely reported.

Background: primary sclerosing cholangitis is a rare and progressive cholestatic disease characterized by narrowing of the biliary tract. Despite high levels of ulcerative colitis, early diagnosis of primary sclerosing cholangitis before the diagnosis of inflammatory bowel disease is rarely reported. Case history: A 28-year-old woman with no history of medical disease visited our clinic because of a mild generalized pruritus. In initial blood laboratory tests with elevated liver enzymes was observed. In Magnetic resonance cholangiopancreatography (MRCP ) was introduced primary sclerosing cholangitis to the patient. Therefore, the patient was under colonoscopy and extensive colitis with terminal ileum involvement was detected and the diagnosis of ulcerative colitis was confirmed in the pathology. Conclusion: Patients diagnosed with primary sclerosing cholangitis should undergo biopsy colonoscopy to rule out inflammatory bowel disease, even if they have no digestive tract symptoms.