New guidelines for screening,evaluation and management of hepatobiliary disease in cystic fibrosis  

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作  者:P.Aiden McCormick Emer Fitzpatrick 

机构地区:[1]Liver Unit,St Vincent’s University Hospital,Dublin,Ireland [2]Department of Medicine,UCD School of Medicine,University College Dublin,Dublin,Ireland [3]Gastroenterology and Liver Department,Children’s Health Ireland,Crumlin,Dublin,Ireland

出  处:《Hepatobiliary Surgery and Nutrition》2024年第5期875-878,共4页肝胆外科与营养(英文)

摘  要:The recently published guidelines on screening,monitoring and treatment update previous guidelines published 25 years ago(1,2).This update is timely as much has happened in the interim.Cystic fibrosis(CF)modulator therapy has dramatically improved prognosis for patients with CF,although it is not yet clear whether liver disease is improved.Our understanding of the pathophysiology of CF liver disease has also changed with the recognition of the importance of non-cirrhotic portal hypertension(3,4).Non-invasive tests for fibrosis and elastography have changed the practice of hepatology and reduced the need for liver biopsy.The guideline committee was made up of experts from North America and Europe and included adult and paediatric hepatologists and pulmonologists together with allied health practitioners and representatives of the CF community.A systematic literature search was performed and a vote of 80%was required to adopt a recommendation.The guidelines contain 7 recommendations for screening,13 for disease monitoring and 14 for treatment.

关 键 词:CIRRHOSIS oesophageal varices non-cirrhotic portal hypertension porto-sinusoidal vascular disease HEPATOBILIARY 

分 类 号:R575[医药卫生—消化系统]

 

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