Abstract

Summary

The advent of enhanced radiological imaging techniques has facilitated the diagnosis of cystic liver lesions. Concomitantly, the evidence base supporting the management of these diseases has matured over the last decades. As a result, comprehensive clinical guidance on the subject matter is warranted. These Clinical Practice Guidelines cover the diagnosis and management of hepatic cysts, mucinous cystic neoplasms of the liver, biliary hamartomas, polycystic liver disease, Caroli disease, Caroli syndrome, biliary hamartomas and peribiliary cysts. On the basis of 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 cystic liver disease.

ABSTRACT

Background and Aim

Direct cholangioscopy and pancreatoscopy have become widely implemented techniques in the diagnostic and therapeutic algorithms of several pancreaticobiliary disorders. This study aimed to generate general and indication-specific European consensus recommendations on cholangioscopy and pancreatoscopy.

Methods

Supported by the available literature, statements were formulated and grouped into the following categories: (1) pre-procedural considerations, (2) general technical aspects, (3) biliopancreatic stones, (4) biliary strictures, and (5) other indications. The evidence level of each statement was determined using the GRADE methodology. Cholangioscopy experts were invited to participate in a modified Delphi process. When 80% consensus was not reached, the statement was modified based on expert feedback and subjected to an additional Delphi round. Statements were rejected if they failed to reach consensus after three Delphi rounds.

Results

Thirty cholangioscopy experts completed the Delphi process. Forty-two (97.6%) generated statements were accepted, of which 39 (92.9%) in the first Delphi round. 12 statements on preprocedural and periprocedural settings, 8 statements on biliopancreatic stones, 13 statements on biliary strictures, and 9 statements on other indications were accepted.

Conclusion

Using a modified Delphi process, we developed general and indication-specific consensus recommendations for cholangioscopy to guide clinical practice.

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

Abstract

Introduction

Eosinophilic esophagitis (EoE) is a chronic inflammatory disease of the esophagus characterized by symptoms of esophageal dysfunction and histologically by predominantly eosinophilic infiltration of the squamous epithelium. European Society for Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) published a guideline in 2014; however, the rapid evolution of knowledge about pathophysiology, diagnostic criteria, and therapeutic options have made an update necessary.

Methods

A consensus group of pediatric gastroenterologists from the ESPGHAN Working Group on Eosinophilic Gastrointestinal Diseases (ESPGHAN EGID WG) reviewed the recent literature and proposed statements and recommendations on 28 relevant questions about EoE. A comprehensive electronic literature search was performed in MEDLINE, EMBASE, and Cochrane databases from 2014 to 2022. The Grading of Recommendations Assessment, Development and Evaluation system was used to assess the quality of evidence and formulate recommendations.

Results

A total of 52 statements based on the available evidence and 44 consensus-based recommendations are available. A revision of the diagnostic protocol, options for initial drug treatment, and the new concept of simplified empiric elimination diets are now available. Biologics are becoming a part of the potential armamentarium for refractory EoE, and systemic steroids may be considered as the initial treatment for esophageal strictures before esophageal dilation. The importance and assessment of quality of life and a planned transition to adult medical care are new areas addressed in this guideline.

Conclusion

Research in recent years has led to a better understanding of childhood EoE. This guideline incorporates the new findings and provides a practical guide for clinicians treating children diagnosed with EoE.