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Background: We conducted a randomized controlled trial to evaluate the clinical efficacy and safety of intravenous doripenem (DRPM) in patients with biliary tract infection requiring biliary drainage in comparison with imipenem/cilastatin (IPM/CS)., Methods: After the initial collection of bile, patients were randomly assigned by the registration system of the Clinical Research Organization to receive intravenous drip infusion of DRPM 0.5 g or IPM/CS 0.5 g three times daily in a randomized, open-label manner., Results: A total of 127 patients were enrolled in the trial (DRPM 62, IPM/CS 65). The characteristics of the 122 patients evaluated for efficacy were well balanced, except for the percentage of patients previously receiving antimicrobials, which was higher in the DRPM group than in the IPM/CS group. The clinical response rate was not significantly different between the DRPM group (93.1 %, 54/58 patients) and the IPM/CS group (93.8 %, 60/64). Non-inferiority assessment using confidence intervals demonstrated the non-inferiority of DRPM-IPM/CS. The incidence of adverse events, for which a causal relationship with either treatment was not ruled out, was 3.3 % (2/60) in the DRPM group and 3.1 % (2/65) in the IPM/CS group, and none was serious., Conclusions: The clinical efficacy of DRPM in treating moderate or severe biliary tract infection requiring drainage was comparable to that of IPM/CS. DRPM was associated with no serious adverse events and a low incidence of adverse events. The results of this trial demonstrate that DRPM is a useful therapeutic option for moderate or severe biliary tract infection.

The present study investigated the possible risk factors, including relationship/HLA matching between donor and recipient, and immunosuppressive therapies on the recurrence of primary sclerosing cholangitis (PSC) after liver transplantation (LT). Subjects were 197 recipients of LT for PSC, among whom 180 surviving more than 1 year after LT were further analyzed for risk factors of recurrence. The 5‐ and 10‐year patient‐ and graft survival rates were 83% and 68%, and 71% and 62%, respectively. The overall PSC recurrence rate was 25% with a 5‐ and 10‐year graft survival rate of 34% and 18%, which was significantly lower than the survival rate of those without recurrence (P < 0.001). Univariate analysis identified the following as risk factors for recurrence: donor age (P < 0.001), cyclosporine use (P = 0.012), mono or no immunosuppressive agent (P < 0.001), postoperative biliary complication (P < 0.001), and active intestinal bowel disease after LT (P < 0.001). Among these factors, donor age ≥45 years [hazard ratio (HR), 1.65; 95% confidence interval (CI), 1.21–2.69; P = 0.003] and mono or no immunosuppressive agent 1‐year after LT (HR, 2.38; 95% CI, 1.23–3.45; P = 0.011) were identified as independent risk factors in the final multivariate Cox regression model. The results were similar in sub‐analysis for ABO‐identical/compatible adult living donor LT cases. [ABSTRACT FROM AUTHOR]

Aim: Primary sclerosing cholangitis (PSC) is very rare in Japan. Although a large‐scale cohort study of 781 pediatric‐onset PSC patients in Europe and North America showed that the 5‐year survival with native liver was 88%, the long‐term outcomes of pediatric‐onset PSC in Japan are unknown. Here, we evaluated the clinical outcomes of pediatric‐onset PSC in Japan. Methods: We carried out a retrospective cohort study with a medical records review of pediatric PSC patients diagnosed between 1986 and 2017 at a single center. The PSC diagnoses were based on cholangiography, liver histology, and biochemical findings. The patients' survival was analyzed using the Kaplan–Meier method. Prognostic factors were determined by univariate and multivariate analyses using the Cox proportional hazards regression model. Results: We identified 39 pediatric‐onset PSC patients (22 boys, 17 girls). The median age at diagnosis was 9 years (interquartile range 6.0–13.5 years). The median follow‐up period was 5.5 years (interquartile range 3.4–8.7 years). The phenotypes of PSC‐autoimmune hepatitis, PSC‐inflammatory bowel disease, and small‐duct PSC were diagnosed in 13 (33.3%), 36 out of 38 (94.8%), and three (7.7%) patients, respectively. The 5‐year liver transplantation‐free survival of the whole cohort was 93.5%. Nine patients underwent liver transplantation, and four of these nine cases resulted in death. Both the univariate and multivariate analyses showed that the phenotype of "PSC‐autoimmune hepatitis overlap" was an independent poor prognostic factor. Conclusions: The overall survival of pediatric‐onset PSC in Japan was comparable to those in Western countries. The phenotype of PSC‐autoimmune hepatitis was identified as a prognostic factor associated with a poorer long‐term outcome. [ABSTRACT FROM AUTHOR]

Aim: Autoimmune liver diseases (AILD) including autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) impose a significant burden on public health, and it is important to estimate their prevalence. We carried out a nationwide, hospital‐based, epidemiological survey of AIH, PBC, and PSC, and compared the results with those from previous surveys. Method: We randomly selected health‐care facilities used in the survey from a list of all facilities in Japan. The selection rate was determined according to a stratification based on the facility characteristics and scale. We sent questionnaires to the selected facilities enquiring about the number and sex of patients with AILD who visited the facility in 2016. An identical survey was undertaken for AIH/PBC in 2004 and for PSC in 2007; we carried out a comparative analysis of these data. Results: We selected 1793 departments from health centers all over Japan. Of them, 1078 (60.1%) responded to the questionnaires. The number of reported patients with AIH, PBC, and PSC was 8505, 10 847, and 906, respectively, and point prevalence was 23.9 (95% confidence interval, 23.3–24.5) for AIH (8.7 in 2004), 33.8 (33.0–34.6) for PBC (11.6 in 2004), and 1.80 (1.75–1.85) for PSC (0.95 in 2007) per 100 000 population. Male‐to‐female patient ratio of AILD was 1:4.3 for AIH (1:6.9 in 2004), 1:3.9 for PBC (1:7.1 in 2004), and 1:0.88 for PSC (1:1.4 in 2007). Conclusion: The current study indicates an increasing trend of prevalence as well as male‐to‐female patient ratio of AILD in Japan. [ABSTRACT FROM AUTHOR]

Background: Primary sclerosing cholangitis (PSC) is relatively rare disease and pathogenesis and methods of treatments were still not established. Then, we had conducted the making clinical guidelines to manage patients with PSC based on the literature review and expert opinions. These clinical guidelines were made for the medical doctors on the management of PSC, except child case of PSC.Methods: We had employed modified Delphi method. The production committee decided guidelines, strength of recommendations and evidence level after reviewed literatures systematically, and The Expert panel evaluated those. The Scientific Committee of the Japan Biliary Association (JBA) evaluated revised guidelines, and the Public comments were collected on web site of JBA.Results: We had made 16 guidelines about epidemiology/pathophysiology, diagnostics, therapy and prognosis. Also, we had made both diagnostic and therapeutic flow chart.Conclusions: We hope that these guidelines will contribute to the improvement and development of the medical care of PSC. [ABSTRACT FROM AUTHOR]

The article "Clinical guidelines for primary sclerosing cholangitis 2017", written by Hiroyuki Isayama, Susumu Tazuma, Norihiro Kokudo, Atsushi Tanaka, Toshio Tsuyuguchi, Takahiro Nakazawa, Kenji Notohara, Suguru Mizuno. [ABSTRACT FROM AUTHOR]

Background: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown cause that is characterized pathologically by an inflammatory and fibrotic process centered on the epithelium, leading to diffuse biliary stenosis and increased wall thickness throughout the intra- and extra-hepatic biliary trees. A nationwide survey in Japan revealed several differences in the clinical aspects of PSC between Japan and Western countries. PSC was recently designated an intractable hepatobiliary disease in Japan. The aim of this study was to establish Japanese diagnostic criteria for PSC according to the current Japanese conditions.Methods: The Intractable Hepato-Biliary Diseases Study Group in Japan of the Committee of Research on Measures for Intractable Diseases established a working group consisting of researchers specializing in PSC and proposed the 2016 Japanese diagnostic criteria for PSC.Results: The diagnostic criteria consisted of the major and minor items. Major items are directly related to bile duct changes based on biliary tract imaging and an increased alkaline phosphatase level. Minor items consist of an association with inflammatory bowel disease and liver histology.Conclusion: The diagnostic criteria for PSC are useful for general physicians in practice. [ABSTRACT FROM AUTHOR]