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Background & Aims: Primary sclerosing cholangitis (PSC) is a progressive, cholestatic liver disease which greatly impacts the lives of individuals. Burden of disease due to shortened life expectancy and impaired quality of life is ill-described. The aim of this study was to assess long-term disease burden in a large population-based registry with regard to survival, clinical course, quality adjusted life years (QALYs), medical consumption and work productivity loss. Methods: All PSC patients living in a geographically defined area covering similar to 50% of the Netherlands were included, together with patients from the three liver transplant centres. Survival was estimated by competing risk analysis. Proportional shortfall of QALYs during disease course was measured relative to a matched reference cohort using validated questionnaires. Work productivity loss and medical consumption were evaluated over time. Results: A total of 1208 patients were included with a median follow-up of 11.2 year. Median liver transplant-free survival was 21.0 years. Proportional shortfall of QALYs increased to 48% >25 years after diagnosis. Patients had on average 12.4 hospital contact days among which 3.17 admission days per year, annual medical costs were (sic)12 169 and mean work productivity loss was 25%. Conclusions: Our data quantify for the first time disease burden in terms of QALYs lost, clinical events, medical consumption, costs as well as work productivity loss, and show that all these are substantial and increase over time.

This report analyses the evolution and the role of industrial relations in the public sector in the Netherlands in the period 2000-2015, focussing on three sub-sectors: hospitals, primary education and municipalities and their task of re-integrating jobseekers. After introducing the Dutch public sector, public sector reforms, and industrial relations, the parts on sub-sectors discuss 1) changes in industrial relations and the shape of public sector reform; 2) the influence of social partners on reform processes and implementation; and 3) effects of reforms on employment and, in turn, effects of the latter on availability and quality of public services. The role of the financial and economic crisis is a recurring theme in addressing these topics. Our in-depth analysis of the various sub-sectors is based on statistical data, documents and interviews with social partners and policy makers. We find that for hospitals, municipalities, and primary education, the landscape of actors has remained stable overall. However, patterns of interaction of the social partners varied from somewhat more consensual in the hospital sector to less consensual in municipalities where collective agreement negotiations were long-drawn and sometimes got held up by industrial action. The latter was also the case in the primary education sector, which recently saw widespread collective action. Furthermore, all sub-sectors experienced major systemic changes between 2000-2015, although the speed differed: municipalities saw stepwise reforms in re-integration and a large reform in 2015. Hospitals were confronted with systemic health system change in 2006. In primary education, schools’ financing method changed to lump-sum financing. Social partners mainly used lobbying strategies towards the central government, however, with differences in intensity between employers and trade unions across sub-sectors and over time. Next, effects of reforms on employment included mixed developments in terms of employment numbers and consequences in terms of e.g. higher work pressure and crisis-induced zero-wage policies in some sub-sectors. Finally, effects of changes in employment on public service provision are hard to establish because of other influences, but the risk of deterioration of services is recognized by especially trade unions despite notable efforts to increase public transparency of quality, for instance in hospitals, and professionalization, for instance in municipal re-integration services.

Item does not contain fulltext Extensive population-based studies are much needed to accurately establish epidemiology and disease course in patients with primary sclerosing cholangitis (PSC). We aimed to obtain population-based prevalence and incidence figures, insight in disease course with regard to survival, liver transplantation (LT), and occurrence of malignancies, as well as risk factors thereof. Four independent hospital databases were searched in 44 hospitals in a large geographically defined area of the Netherlands, comprising 50% of the population. In addition, all PSC patients in the three Dutch liver transplant centers and all inflammatory bowel disease (IBD) patients in the adherence area of a large district hospital were identified. All medical records were reviewed on-site, verifying diagnosis. Five hundred and ninety PSC patients were identified, resulting in an incidence of 0.5 and a point prevalence of 6.0 per 100,000. Median follow up was 92 months. Estimated median survival from diagnosis until LT or PSC-related death in the entire cohort was 21.3 years, as opposed to 13.2 years in the combined transplant centers cohort (n = 422; P < 0.0001). Colorectal carcinoma (CRC) risk was 10-fold increased, as compared to ulcerative colitis controls, and developed at a much younger age (39 years; range, 26-64), compared to IBD controls (59 years; range, 34-73; P = 0.019). Colonoscopic surveillance was associated with significantly better outcome. Conclusion: This study exemplifies that, for relatively rare diseases, it is paramount to collect observational data from large, population-based cohorts, because incidence and prevalence rates of PSC are markedly lower and survival much longer than previously reported. The selection of a bias-free, population-based cohort showed a significantly longer survival, compared to the tertiary referral cohort. CRC can develop at an early age, warranting surveillance from time of PSC diagnosis. (Hepatology 2013; 58:2045-2055).

BACKGROUND: Criteria assessing biochemical response to ursodeoxycholic acid (UDCA) are established risk stratification tools in primary biliary cholangitis (PBC). We aimed to evaluate to what extent liver tests influenced patient management during a three decade period, and whether this changed over time. METHODS: 851 Dutch PBC patients diagnosed between 1988 and 2012 were reviewed to assess patient management in relation to liver test results during UDCA treatment. To do so, biochemical response at one year was analysed retrospectively according to Paris-1 criteria. RESULTS: Response was assessable for 687/851 (81%) patients; 157/687 non-responders. During a follow-up of 8.8 years (IQR 4.8-13.9), 141 died and 30 underwent liver transplantation. Transplant-free survival of non-responders (60%) was significantly worse compared with responders (87%) (p < 0.0001). Management was modified in 46/157 (29%) non-responders. The most frequent change observed, noted in 26/46 patients, was an increase in UDCA dosage. Subsequently, 9/26 (35%) non-responders became responders within the next two years. Steroid treatment was started in one patient; 19 patients were referred to a tertiary centre. No trend towards more frequent changes in management over time was observed (p = 0.10). CONCLUSION: Changes in medical management occurred in a minority of non-responders. This can largely be explained by the lack of accepted response criteria and of established second-line treatments for PBC. Nevertheless, the observation that response-guided management did not increase over time suggests that awareness of the concept of biochemical response requires further attention,particularly since new treatment options for PBC will soon become available.

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Primary sclerosing cholangitis (PSC) is a cholestatic liver disease of unknown cause, with genetic predisposition in combination with environmental factors possibly playing a role.- The diagnosis is made on the basis of a liver enzyme profile indicating cholestasis and characteristic bile duct abnormalities in cholangiography or the liver biopsy after excluding other causes.- Approximately 80% of patients have concurrent inflammatory bowel disease (IBD), specifically ulcerative colitis in most patients.- PSC predisposes to hepatobiliary malignancies such as cholangiocarcinoma, gallbladder carcinoma and hepatocellular carcinoma, as well as to colorectal carcinoma in patients with concurrent IBD.- UDCA and endoscopic bile duct dilatation relieve symptoms and improve the liver enzyme profile.- Orthotopic liver transplantation is the only potentially curative therapy available