Summary

Acute-on-chronic liver failure (ACLF), which was described relatively recently (2013), is a severe form of acutely decompensated cirrhosis characterised by the existence of organ system failure(s) and a high risk of short-term mortality. ACLF is caused by an excessive systemic inflammatory response triggered by precipitants that are clinically apparent (e.g., proven microbial infection with sepsis, severe alcohol-related hepatitis) or not. Since the description of ACLF, some important studies have suggested that patients with ACLF may benefit from liver transplantation and because of this, should be urgently stabilised for transplantation by receiving appropriate treatment of identified precipitants, and full general management, including support of organ systems in the intensive care unit (ICU). The objective of the present Clinical Practice Guidelines is to provide recommendations to help clinicians recognise ACLF, make triage decisions (ICU vs. no ICU), identify and manage acute precipitants, identify organ systems that require support or replacement, define potential criteria for futility of intensive care, and identify potential indications for liver transplantation. Based on an in-depth review of the relevant literature, we provide recommendations to navigate clinical dilemmas followed by supporting text. The recommendations are graded according to the Oxford Centre for Evidence-Based Medicine system and categorised as ‘weak’ or ‘strong’. We aim to provide the best available evidence to aid the clinical decision-making process in the management of patients with ACLF.

Keywords

Organ failure, inflammation, precipitating event, infection, alcohol-related hepatitis, critical care, organ support, sarcopenia, frailty, liver,, transplantation, prognosis, futility, mortality

Summary

Wilson’s disease is an autosomal recessive disorder of copper metabolism which affects the liver, brain and other organs. Diagnosis is based on: clinical features; biochemical tests, including plasma ceruloplasmin concentration, 24-h urinary copper excretion, copper content in the liver; and molecular analysis. Leipzig score and additionally relative exchangeable copper determination are recommended for diagnosis. Pharmacological therapy comprises chelating agents (penicillamine, trientine) and zinc salts, while only chelators are recommended for significant liver disease. Monitoring is based on clinical symptoms, liver tests and copper metabolism (urinary copper excretion, exchangeable copper) to detect poor compliance and over/under-treatment. Acute liver failure is challenging as making a diagnosis is difficult and pharmacological therapy may not be sufficient to save life. Liver transplantation has a well-defined role in Wilsonian acute hepatic failure but may also be considered in neurological disease.

Abstract

Pancreatic exocrine insufficiency (PEI) is defined as a reduction in pancreatic exocrine secretion below the level that allows the normal digestion of nutrients. Pancreatic disease and surgery are the main causes of PEI. However, other conditions and upper gastrointestinal surgery can also affect the digestive function of the pancreas. PEI can cause symptoms of nutritional malabsorption and deficiencies, which affect the quality of life and increase morbidity and mortality. These guidelines were developed following the United European Gastroenterology framework for the development of high-quality clinical guidelines. After a systematic literature review, the evidence was evaluated according to the Oxford Center for Evidence-Based Medicine and the Grading of Recommendations Assessment, Development, and Evaluation methodology, as appropriate. Statements and comments were developed by the working groups and voted on using the Delphi method. The diagnosis of PEI should be based on a global assessment of symptoms, nutritional status, and a pancreatic secretion test. Pancreatic enzyme replacement therapy (PERT), together with dietary advice and support, are the cornerstones of PEI therapy. PERT is indicated in patients with PEI that is secondary to pancreatic disease, pancreatic surgery, or other metabolic or gastroenterological conditions. Specific recommendations concerning the management of PEI under various clinical conditions are provided based on evidence and expert opinions. This evidence-based guideline summarizes the prevalence, clinical impact, and general diagnostic and therapeutic approaches for PEI, as well as the specifics of PEI in different clinical conditions. Finally, the unmet needs for future research are discussed.

Abstract

Objectives

To provide a consensus statement and recommendations on MR imaging in primary sclerosing cholangitis (PSC).

Methods

The European Society of Gastrointestinal and Abdominal Radiology (ESGAR) convened a multinational European panel of experts selected based on a literature review and their leadership in the field. A modified Delphi process was adopted to draft a list of statements. For each statement, the panelists indicated the level of agreement using a 5-point Likert scale, where 1 means “no agreement,” 2 means “poor agreement,” 3 means “slight agreement,” 4 means “fair agreement,” and 5 means “complete agreement.” The median score for each statement was collected. The level of evidence was reported according to the Oxford Centre for Evidence-Based Medicine. Descriptive statistics were used to rate agreement levels and the consensus’ internal reliability.

Results

The 12 voting committee members were from Italy (n = 4, 33.4%), Austria (n = 2, 16.7%), Sweden (n = 1, 8.3%), France (n = 1, 8.3%), the United States (n = 1, 8.3%), Switzerland (n = 1, 8.3%), and Belgium (n = 2, 16.7%). The final questionnaire consisted of 55 statements. The agreement reached by the expert panel was complete for 23 statements (41.8%), fair for 16 (29.1%), slight for 15 (27.2%), and poor for 1 (1.9%). Statements that received complete agreement were used to structure a reporting template.

Conclusions

This statement paper recommends how and when to perform MRI in PSC patients. A structured reporting template has been created to improve quality care and communication among radiologists and clinicians.

Key Points

Question A standard MR protocol and the most common imaging features to be reported are fundamental for the correct evaluation of primary sclerosing cholangitis (PSC) patients.

Findings Twelve expert radiologists reported which are the most important imaging features and how and when to perform MR in PSC patients.

Clinical relevance The identified statements reported in this paper and the structured reporting template are useful for radiologists and clinicians to help correctly manage PSC patients.

INTRODUCTION

Diet is gaining recognition amongst clinicians, researchers, and the inflammatory bowel disease [IBD] community as a potential modifier to change the course of the disease. The role of diet in the management of IBD has evolved beyond adjustment for nutritional needs and is now applied as therapy to influence disease activity and complications. With growing recognition of diet, there is a demand for high-quality dietary research to guide practice and consideration on where to best position diet in the treatment paradigm. This has led to the commissioning of this first European Crohn’s and Colitis Organisation [ECCO] consensus on dietary management of IBD, with the goal of providing evidence-based recommendations to guide clinical practice.

ABSTRACT

Introduction

Inflammatory bowel disease (IBD), which includes Crohn’s disease (CD) and ulcerative colitis (UC), requires meticulous diagnostic and monitoring protocols to optimize patient outcomes. This document synthesizes key advancements and recommendations for clinical indices, endoscopic scoring, imaging, histological assessment, and tools for evaluating IBD disability. Emphasizing validated, reproducible methods aligns with the ongoing evolution of precision medicine in IBD care, including training. The methodology of the guidelines update process is described in part 1.