Summary

Background

Nutritional status is paramount in Cystic Fibrosis (CF) and is directly correlated with morbidity and mortality. The first ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with CF were published in 2016. An update to these guidelines is presented.

Methods

The study was developed by an international multidisciplinary working group in accordance with officially accepted standards. Literature since 2016 was reviewed, PICO questions were discussed and the GRADE system was utilized. Statements were discussed and submitted for on-line voting by the Working Group and by all ESPEN members.

Results

The Working Group updated the nutritional guidelines including assessment and management at all ages. Supplementation of vitamins and pancreatic enzymes remains largely the same. There are expanded chapters on pregnancy, CF-related liver disease, and CF-related diabetes, bone disease, nutritional and mineral supplements, and probiotics. There are new chapters on nutrition with highly effective modulator therapies and nutrition after organ transplantation.

Keywords

Cystic fibrosis, Nutrition, Guideline update

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.

Summary

Transjugular intrahepatic portosystemic shunt (TIPS) placement is a well-established procedure for managing complications of portal hypertension. In recent years, the field of TIPS has seen rapid advances, particularly in technical approaches, prognostic modelling, and an expanding range of indications. The aim of these EASL guidelines is to comprehensively address all aspects of TIPS in patients with cirrhosis. Other related topics, such as the use of TIPS in surgical settings and in vascular liver disorders, are covered in separate dedicated clinical practice guidelines.

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.

Summary

Autoimmune hepatitis (AIH) is a chronic liver disease of unknown aetiology which may affect any patient irrespective of age, sex, or ethnicity. At baseline, the clinical spectrum of the disease varies largely from asymptomatic cases to acute liver failure with massive hepatocyte necrosis. The aim of these EASL guidelines is to provide updated guidance on the diagnosis and management of AIH both in adults and children. Updated guidance on the management of patients with variants and specific forms of AIH is also provided, as is detailed guidance on the management of AIH-associated cirrhosis, including surveillance for portal hypertension and hepatocellular carcinoma, as well as liver transplantation in decompensated cirrhosis.

Keywords

Autoimmune hepatitis; Autoantibodies; Variant syndromes; Immunosuppression; Azathioprine; Mycophenolate mofetil

ABSTRACT

Executive Summary

Robotic surgery has been utilized increasingly, including in colorectal surgery. Newer robotic platforms are coming onto the market, and more emphasis is being placed on the safety and adequate training of surgeons and theatre teams. Training in robotic colorectal surgery has not been standardized, and there are no agreed structured training and assessment methods. Some studies in minimally invasive surgery across specialities have shown that training curricula shortened the learning curve in minimally invasive surgery and, therefore, there is a greater need for guidance on training in robotic colorectal surgery based on up-to-date available evidence on the subject.

The European Society of Coloproctology (ESCP) Guidelines Committee aimed to conduct a comprehensive literature review, assess currently available evidence and collate expert opinion on training in robotic colorectal surgery. Evidence was graded, and the recommendation was based on the GRADE (Grading of Recommendations Assessment, Development and Evaluation) methodology. When evidence is lacking expert opinion is considered, and the research gap is highlighted.