Towards a Standardized Classification of the Hepatobiliary Manifestations in Cystic Fibrosis (CFHBI): A Joint ESPGHAN/NASPGHAN Position Paper

Author:

Bodewes Frank A.J.A.1,Freeman Alvin Jay2,Weymann Alexander3,Debray Dominique4,Scheers Isabelle5,Verkade Henkjan J.1,Narkewicz Michael R.6

Affiliation:

1. Division of Pediatric Gastroenterology/Hepatology, Beatrix Children's Hospital/University Medical Center Groningen University of Groningen Groningen Netherlands

2. Division of Gastroenterology, Hepatology and Nutrition, Children's Healthcare of Atlanta Emory University Atlanta GA

3. Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics Nationwide Children's Hospital/The Ohio State University College of Medicine Columbus OH

4. Pediatric Hepatology Unit, Assistance Publique‐Hôpitaux de Paris (APHP)‐Hôpital Necker‐Enfants maladies Sorbonne Université, INSERM, Centre de Recherche Saint‐Antoine (CRSA) Paris France

5. Pediatric Gastroenterology, Hepatology and Nutrition Unit, Cliniques Universitaires Saint‐Luc Université Catholique de Louvain Brussels Belgium

6. Digestive Health Institute, Children's Hospital Colorado and Section of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics University of Colorado School of Medicine Aurora CO

Abstract

AbstractThe broad spectrum of hepatobiliary involvement in cystic fibrosis (CF) has been commonly referred to as cystic fibrosis liver disease (CFLD). However, differences in the definitions of CFLD have led to variations in reported prevalence, incidence rates, and standardized recommendations for diagnosis and therapies.Harmonizing the description of the spectrum of hepatobiliary involvement in all people with CF (pwCF) is deemed essential for providing a reliable account of the natural history, which in turn supports the development of meaningful clinical outcomes in patient care and research.Recognizing this necessity, The European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) and the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) commissioned and tasked a committee to develop and propose a systematic classification of the CF hepatobiliary manifestations to increase uniformity, accuracy, and comparability for clinical, registry, and research purposes. This report describes the committee's combined expert position statement on hepatobiliary involvement in CF, which has been endorsed by NASPGHAN and ESPGHAN.We recommend using CFHBI (Cystic Fibrosis Hepato‐Biliary Involvement) as the updated term to describe and classify all hepatobiliary manifestations in all pwCF. CFHBI encompasses the current extensive spectrum of phenotypical, clinical, or diagnostic expressions of liver involvement observed in pwCF. We present a schematic categorization of CFHBI, which may also be used to track and classify the changes and development of CFHBI in pwCF over time. The proposed classification for CFHBI is based on expert consensus and has not been validated for clinical practice and research purposes. Achieving validation should be an important aim for future research.

Publisher

Wiley

Reference108 articles.

1. Cystic Fibrosis–related Liver Disease: Research Challenges and Future Perspectives

2. Prevalence of elevated liver function tests in children with cystic fibrosis diagnosed by newborn screen;Woodruff SA;J Pediatr Gastroenterol Nutr,2007

3. Ursodeoxycholic acid for cystic fibrosis‐related liver disease;Cheng K;Cochrane Database Syst Rev,2017

4. Cystic Fibrosis Liver Disease: Outcomes and Risk Factors in a Large Cohort of French Patients

同舟云学术

1.学者识别学者识别

2.学术分析学术分析

3.人才评估人才评估

"同舟云学术"是以全球学者为主线,采集、加工和组织学术论文而形成的新型学术文献查询和分析系统,可以对全球学者进行文献检索和人才价值评估。用户可以通过关注某些学科领域的顶尖人物而持续追踪该领域的学科进展和研究前沿。经过近期的数据扩容,当前同舟云学术共收录了国内外主流学术期刊6万余种,收集的期刊论文及会议论文总量共计约1.5亿篇,并以每天添加12000余篇中外论文的速度递增。我们也可以为用户提供个性化、定制化的学者数据。欢迎来电咨询!咨询电话:010-8811{复制后删除}0370

www.globalauthorid.com

TOP

Copyright © 2019-2024 北京同舟云网络信息技术有限公司
京公网安备11010802033243号  京ICP备18003416号-3