Nonfasted Liver Stiffness Correlates with Liver Disease Parameters and Portal Hypertension in Pediatric Cholestatic Liver Disease

• Type of Material: Article
• Authors: Benjamin L. Shneider, Baylor College of Medicine; Nathan P. Goodrich, Arbor Research Collaborative for Health; Wen Ye, University of Michigan; Cindy Sawyers, Indiana University; Jean P. Molleston, Indiana University; Robert M. Merion, Arbor Research Collaborative for Health; Daniel H. Leung, Baylor College of Medicine; Saul Karpen, Emory University; Binita M. Kamath, Hospital for Sick Children; Laurel Cavallo, Baylor College of Medicine; Kasper Wang, Childrens Hospital Los Angeles; Jeffrey H. Teckman, St Louis University; James E. Squires, UPMC Childrens Hospital Pittsburgh; Shikha S. Sundaram, University of Colorado; Philip Rosenthal, University of California San Francisco; Rene Romero, Emory University; Karen F. Murray, Seattle Childrens Hospital; Kathleen M. Loomes, University of Pennsylvania; M. Kyle Jensen, Primary Childrens Medical Center; Jorge A. Bezerra, Cincinnati Childrens Hospital Medical Center; Lee M. Bass, Ann & Robert H Lurie Children's Hospital; Ronald J. Sokol, University of Colorado; John C. Magee, University of Michigan
• Language: English
• Date: 2020-08-05
• Publisher: JOHN WILEY & SONS LTD
• Publication Version: Final Published Version
• Copyright Statement: © 2020 The Authors. Hepatology Communications published by Wiley Periodicals LLC on behalf of the American Association for the Study of Liver Diseases.
• License: Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1002/hep4.1574
• Title of Journal or Parent Work: HEPATOLOGY COMMUNICATIONS
• Volume: 4
• Issue: 11
• Start Page: 1694
• End Page: 1707
• Grant/Funding Information: Supported by the National Institute of Diabetes and Digestive and Kidney Diseases (grants DK62436 to Ann and Robert H. Lurie Children's Hospital of Chicago, DK62497 to Cincinnati Children's Hospital Medical Center, DK62453 to Children's Hospital Colorado, DK62481 to Children's Hospital of Philadelphia, DK62466 to Children's Hospital of Pittsburgh, DK62500 to University of California San Francisco (UCSF) Children's Hospital, DK62453 to St. Louis University School of Medicine, DK84536 to Riley Hospital for Children of Indiana University School of Medicine, DK84575 to Seattle Children's Hospital, DK103135 to The Hospital for Sick Children, DK103140 to University of Utah, DK84538 to Children's Hospital Los Angeles, DK062470 to Children's Healthcare of Atlanta, DK103149 to Texas Children's Hospital, DK62456 to Scientific Data Coordinating Center), the National Center for Advancing Translational Sciences (grants UL1TR001422 to Ann and Robert H. Lurie Children's Hospital of Chicago, UL1TR000077 to Cincinnati Children's Hospital Medical Center, UL1TR002535 to Children's Hospital Colorado, UL1TR000005 to Children's Hospital of Pittsburgh, UL1TR000004 to UCSF Children's Hospital, UL1TR001108 to Riley Hospital for Children at Indiana University School of Medicine, UL1TR000423 to Seattle Children's Hospital, UL1TR000130 to Children's Hospital Los Angeles, UL1TR000454 to Children's Healthcare of Atlanta), and King's College Hospital, London (grant XXX).
• Keywords: CHILDREN; Science & Technology; Life Sciences & Biomedicine; Gastroenterology & Hepatology; FIBROSIS; TRANSIENT ELASTOGRAPHY; HEPATITIS-C
• Research Categories: Health Sciences, Pathology; Health Sciences, Medicine and Surgery

Abstract

Elastographic measurement of liver stiffness is of growing importance in the assessment of liver disease. Pediatric experiences with this technique are primarily single center and limited in scope. The Childhood Liver Disease Research Network provided a unique opportunity to assess elastography in a well-characterized multi-institutional cohort. Children with biliary atresia (BA), alpha-1 antitrypsin deficiency (A1ATD), or Alagille syndrome (ALGS) followed in a prospective longitudinal network study were eligible for enrollment in a prospective investigation of transient elastography (FibroScan). Studies were performed in participants who were nonfasted and nonsedated. Liver stiffness measurements (LSMs) were correlated with standard clinical and biochemical parameters of liver disease along with a research definition of clinically evident portal hypertension (CEPH) graded as absent, possible, or definite. Between November 2016 and August 2019, 550 participants with a mean age of 8.8 years were enrolled, 458 of whom had valid LSMs (BA, n = 254; A1ATD, n = 104; ALGS, n = 100). Invalid scans were more common in participants <2 years old. There was a positive correlation between LSM and total bilirubin, international normalized ratio (INR), aspartate aminotransferase (AST), alanine aminotransferase (ALT), gamma-glutamyl transpeptidase (GGT), GGT to platelet ratio (GPR), pediatric end-stage liver disease score, AST to platelet ratio index, and spleen size, and a negative correlation with albumin and platelet count in BA, with similar correlations for A1ATD (except AST, ALT, and albumin) and ALGS (except for INR, GGT, GPR, and ALT). Possible or definite CEPH was more common in BA compared to ALGS and A1ATD. LSM was greater in definite versus absent CEPH in all three diseases. Disease-specific clinical and biochemical characteristics of the different CEPH grades were observed. Conclusion: It is feasible to obtain LSMs in children, especially over the age of 2 years. LSM correlates with liver parameters and portal hypertension, although disease-specific patterns exist.