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Background and Aims: Primary sclerosing cholangitis (PSC) is a chronic cholestatic disease that causes liver cirrhosis, leading to liver failure. Additionally, PSC is a risk factor for cholangiocarcinoma. Its mechanism is unknown, and liver transplantation remains the sole curative option. The membrane bound O-acyltransferase domain containing 7 (MBOAT7) rs641738 and rs626283 variant alleles have been associated with both an accelerated progression of the disease and a higher risk for developing a more severe phenotype in many chronic hepatic diseases. Thus, we analysed their effect on long-term outcomes and laboratory parameters in PSC patients., Methods: We determined MBOAT7 genotypes and estimated the actuarial survival rate free of liver transplantation, using the Kaplan-Meier estimator. The differences between the estimates were analysed using the log-rank test. Patient blood was drawn and analysed for different serum parameters including cholestatic markers. Additionally, MBOAT7 RNA expression in human hepatic cell lines MZCHA1 (a biliary adenocarcinoma cell line), HepG2 (a hepatocellular carcinoma cell line), LX-2 (hepatic stellate cell line) and H-69 (cholangiocyte cell line) was analysed., Results: Transplant-free survival was significantly prolonged in carriers of two rs641738 variant alleles, which was referred to as the TT genotype (mean 19.6 years; 95% confidence interval [CI]: 16.3-22.9 years) compared to the CC (mean 15.4 years, 95% CI 12.8-18.0 years) and heterozygous genotypes (mean 13.2 years, 95% CI 11.4-15.0 years) (P=0.017). This effect was restricted to male patients. We confirmed the high expression of MBOAT7 in hepatic stellate cells and found that MBOAT7 is less expressed in biliary epithelial cell lines, compared to parenchymal hepatic cells., Conclusions: Unlike other chronic liver diseases, carrying two MBOAT7 variant alleles does not seem to affect PSC patients negatively, but seems to have a positive effect on transplant-free survival. This study could help improve individual prognosis in PSC patients and give some new perspective on the involvement of the immune system in PSC., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Background: After liver transplantation (LT), biliary complications are associated with reduced graft survival. We tested inflammation markers for their association with biliary damage and graft loss in bile., Material and Methods: The study design was a retrospective case-control study. Calprotectin, lactoferrin and pyruvate kinase were measured in endoscopically retrieved bile with ELISA., Results: Calprotectin and lactoferrin were significantly higher in bile of ischemic-type biliary lesions and donor duct non-anastomotic strictures than in control, bile leakage, Cytomegalovirus infection, anastomotic stricture or acute cellular rejection patients (p<0.001) independent of serum liver values at endoscopy. Calprotectin (p=0.02) was independently associated with retransplantation free survival in multivariate analysis, as was γGT (p=0.03) but not ERC radiographic classification of the bile duct or cold ischemia time., Conclusion: Calprotectin and lactoferrin are bile markers for biliary damage and are associated with re-transplantation free survival. They can differentiate progressive biliary damage from non-biliary liver value alterations after LT., (Copyright © 2019. Published by Elsevier Masson SAS.)

Primary sclerosing cholangitis (PSC) is characterized by a chronic inflammatory process of the bile ducts of unclear aetiology. It is often complicated by cholangiocarcinoma (CCA) with a dismal prognosis. Early detection of CCA is important because treatment options for advanced disease are limited. Besides the established markers, like CA19-9, recent developments have been made using latest technologies. This review summarizes the recent advances and remaining limitations of biomarkers of CCA in PSC., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Background: Wilson disease is an autosomal recessive disorder of copper metabolism and requires lifelong medical treatment. Therefore, the analysis of quality of life has gathered more attention. Aims of this study were to examine risk for depression and health-related quality of life in patients suffering from Wilson disease., Methods: Sixty-eight patients were included in this retrospective cross sectional study. The Personal Health Questionnaire-9 Depression Scale was used to assess depression. The Short Form-36 Health Survey questionnaire was used to assess health-related quality of life., Results: The Personal Health Questionnaire-9 indicated that 21% (14/68) of patients were at risk for major depressive disorders (scores>10) and 35% (24/68) were at risk for mild depression (scores 5-9). Women had significantly lower life quality scores than men. Primary neurologic disease manifestation was associated with significantly lower total Short Form-36 and subdimension scores compared with primary hepatic or mixed presentation. Overall, patients with Wilson disease experienced higher quality of life than patients with other chronic liver diseases., Conclusions: As patients with Wilson disease have a high risk for depressive disorders, active assessment for depression is mandatory. Patients with primary neurological symptoms are at higher risk for reduction of life quality., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)