Liver transplant-free survival according to alkaline phosphatase and GLOBE score in patients with primary biliary cholangitis treated with ursodeoxycholic acid.

Background: After 1 year of ursodeoxycholic acid (UDCA), patients with primary biliary cholangitis (PBC) may have a normal GLOBE score despite high alkaline phosphatase (ALP) levels.

Aim: To assess the association between ALP and liver transplantation (LT)-free survival according to the GLOBE score METHODS: Among patients with a normal or elevated GLOBE score in the Global PBC cohort, the association between ALP after 1 year of UDCA and the risk of LT/death was assessed. The LT-free survival was compared with that of a matched general population.

Blue-collar work is a risk factor for developing IgG4-related disease of the biliary tract and pancreas.

Background & Aims: Immunoglobulin G4-related disease (IgG4-RD) of the biliary tract and pancreas is a fibroinflammatory disease of unknown origin with striking male predominance. We aimed to investigate whether blue-collar work and occupational contaminant exposure are risk factors for IgG4-RD of the biliary tract and pancreas.

Method: We performed an age-/sex-matched case-control study in the largest academic medical centers of the Netherlands.

Development and validation of a prognostic score for long-term transplant-free survival in autoimmune hepatitis type 1.

Background: No prognostic score is currently available for long-term survival in autoimmune hepatitis (AIH) patients.

Objective: The aim of this study was to develop and validate such a prognostic score for AIH patients at diagnosis.

Methods: The prognostic score was developed using uni- & multivariate Cox regression in a 4-center Dutch cohort and validated in an independent 6-center Belgian cohort.

A Comparison of Prognostic Scores (Mayo, UK-PBC, and GLOBE) in Primary Biliary Cholangitis.

Introduction: Comparative data on scores that predict outcome in primary biliary cholangitis (PBC) are scarce. We aimed to assess and compare the prognostic value of the Mayo Risk Score (MRS, 1989 and 1994), UK-PBC score, and GLOBE score in a large international cohort of patients with PBC.

Methods: Ursodeoxycholic acid-treated patients from 7 centers participating in the GLOBAL PBC Study Group were included.

[The buried bumper syndrome].

The buried bumper syndrome (BBS) is a rare complication of percutaneous endoscopic gastrostomy (PEG). Hereby the internal PEG bumper is overgrown by hypertrophic gastric mucosa and embedded into the gastric wall. Most often an endoscopic approach to remove the bumper is successful.

Fibrates for Itch (FITCH) in Fibrosing Cholangiopathies: A Double-Blind, Randomized, Placebo-Controlled Trial.

Background And Aims: Pruritus may seriously impair quality of life in patients with cholestatic diseases such as primary or secondary sclerosing cholangitis (PSC, SSC) and primary biliary cholangitis (PBC). Pharmacologic strategies show limited efficacy and can provoke serious side effects. We hypothesized that bezafibrate, a broad peroxisome proliferator-activated receptor (PPAR) agonist, relieves cholestasis-associated itch by alleviating hepatobiliary injury.

Measurement of Gamma Glutamyl Transferase to Determine Risk of Liver Transplantation or Death in Patients With Primary Biliary Cholangitis.

Background & Aims: Gamma-glutamyltransferase (GGT) is a serum marker of cholestasis. We investigated whether serum level of GGT is a prognostic marker for patients with primary biliary cholangitis (PBC).

Methods: We analyzed data from patients with PBC from the Global PBC Study Group, comprising 14 centers in Europe and North America.

Goals of Treatment for Improved Survival in Primary Biliary Cholangitis: Treatment Target Should Be Bilirubin Within the Normal Range and Normalization of Alkaline Phosphatase.

Introduction: In primary biliary cholangitis (PBC), bilirubin and alkaline phosphatase (ALP) are widely established as independent predictors of prognosis. Current treatment goals do not aim for normalization of surrogate markers because their association with survival has not been defined.

Methods: The patient cohort from the GLOBAL PBC Study Group was used, comprising of long-term follow-up data from European and North American centers.

Long-term impact of preventive UDCA therapy after transplantation for primary biliary cholangitis.

Background & Aims: Recurrence of primary biliary cholangitis (PBC) after liver transplantation (LT) is frequent and can impair graft and patient survival. Ursodeoxycholic acid (UDCA) is the current standard therapy for PBC. We investigated the effect of preventive exposure to UDCA on the incidence and long-term consequences of PBC recurrence after LT.

Identification and prognostic impact of malnutrition in a population screened for liver transplantation.

Background & Aims: Sarcopenia is prevalent in patients with liver cirrhosis and is negatively associated with clinical outcomes. In a population screened for liver transplantation we aimed to assess the prevalence of abnormal nutritional status and to what extent a clinical screening tool is able to reliably select patients for extensive nutritional assessment including CT. We also evaluated which nutritional parameters are independently associated with clinical outcomes.

Factors Associated With Progression and Outcomes of Early Stage Primary Biliary Cholangitis.

Background & Aims: Patients usually receive a diagnosis of primary biliary cholangitis (PBC) at an early stage, based on biochemical analyses. We investigated the proportion of patients who progress to moderate or advanced PBC and factors associated with progression and patient survival.

Methods: We obtained data from 1615 patients (mean age, 55.

Validation, clinical utility and limitations of the Amsterdam-Oxford model for primary sclerosing cholangitis.

Background & Aims: Recently the Amsterdam-Oxford model (AOM) was introduced as a prognostic model to assess the risk of death and/or liver transplantation (LT) in primary sclerosing cholangitis (PSC). We aimed to validate and assess the utility of the AOM.

Methods: Clinical and laboratory data were collected from the time of PSC diagnosis until the last visit or time of LT or death.

The efficacy and safety of rifaximin-α: a 2-year observational study of overt hepatic encephalopathy.

Background: After 5  years since the registration of rifaximin-α as a secondary prophylaxis for overt hepatic encephalopathy (HE) in the Netherlands, we aimed to evaluate the use of hospital resources and safety of rifaximin-α treatment in a real-world setting.

Methods: We carried out prospective identification of all patients using rifaximin-α for overt HE. We assessed hospital resource use, bacterial infections, and adverse events during 6-month episodes before and after rifaximin-α initiation.

Ursodeoxycholic acid therapy and liver transplant-free survival in patients with primary biliary cholangitis.

Background & Aims: The clinical efficacy of ursodeoxycholic acid (UDCA) in primary biliary cholangitis (PBC) remains subject to debate as definitive randomized controlled trials are lacking. We aimed to determine whether UDCA prolongs liver transplant (LT)-free survival in patients with PBC.

Methods: This international cohort study included patients from the Global PBC Study Group database, originating from 8 countries in Europe and North America.

The Yield and Safety of Screening Colonoscopy in Patients Evaluated for Liver Transplantation.

Colorectal cancer (CRC) screening with colonoscopy is commonly used in patients who are candidates for liver transplantation. We initiated this study to define the risk-benefit ratio of performing screening colonoscopy in this population. A retrospective observational study of all consecutive patients undergoing colonoscopy during pre-liver transplantation screening between 2004 and 2017 was conducted.

Effects of Age and Sex of Response to Ursodeoxycholic Acid and Transplant-free Survival in Patients With Primary Biliary Cholangitis.

Background & Aims: Primary biliary cholangitis (PBC) predominantly affects middle-aged women; there are few data on disease phenotypes and outcomes of PBC in men and younger patients. We investigated whether differences in sex and/or age at the start of ursodeoxycholic acid (UDCA) treatment are associated with response to therapy, based on biochemical markers, or differences in transplant-free survival.

Methods: We performed a longitudinal retrospective study of 4355 adults in the Global PBC Study cohort, collected from 17 centers across Europe and North America.

Trends in liver transplantation for primary biliary cholangitis in Europe over the past three decades.

Background: The importance of primary biliary cholangitis as an indication for liver transplantation has probably been influenced by the introduction of therapies, and changes in selection criteria and disease epidemiology.

Aims: To assess the time trends in liver transplantation for primary biliary cholangitis and to evaluate the characteristics of the patient population during the past three decades.

Methods: Patients undergoing liver transplantation from 1986 to 2015 in centres reporting to the European Liver Transplantation Registry were included.

Improving prognosis in primary biliary cholangitis - Therapeutic options and strategy.

Overall survival in primary biliary cholangitis is diminished. As patients are often asymptomatic, the disease may silently progress towards cirrhosis and liver failure. Timely diagnosis and effective treatment options are of vital importance to improve the prognosis of affected patients.

Factors Associated With Recurrence of Primary Biliary Cholangitis After Liver Transplantation and Effects on Graft and Patient Survival.

Background & Aims: Primary biliary cholangitis (PBC) frequently recurs after liver transplantation. We evaluated risk factors associated with recurrence of PBC and its effects on patient and graft survival in a multicenter, international cohort (the Global PBC Study Group).

Methods: We collected demographic and clinical data from 785 patients (89% female) with PBC who underwent liver transplantation (mean age, 54 ± 9 years) from February 1983 through June 2016, among 13 centers in North America and Europe.

Increased Mortality Among Patients With vs Without Cirrhosis and Autoimmune Hepatitis.

Background & Aims: There have been few reproducible studies of mortality in patients with autoimmune hepatitis (AIH) and its variants. We calculated mortality in a large national cohort of patients with AIH, with vs without cirrhosis, in the Netherlands.

Methods: We collected data from 449 patients with established AIH (77% female), from 6 academic and 10 non-academic hospitals in the Netherlands.

Variable efficacy of TIPSS in the management of ectopic variceal bleeding: a multicentre retrospective study.

Background: Evidence for the efficacy of TIPSS in ectopic variceal bleeding (EctVB) is largely based on relatively small series.

Aim: To define the efficacy of TIPSS in EctVB.

Methods: Retrospective analysis of consecutive patients with chronic liver disease who presented with EctVB and received TIPSS in three tertiary centres in 1992-2016.