The summaries are free for public
use. The Chronic Liver Disease
Foundation will continue to add and
archive summaries of articles deemed
relevant to CLDF by the Board of
Trustees and its Advisors.
Abstract Details
Nonfasted Liver Stiffness Correlates with Liver Disease Parameters and Portal Hypertension in Pediatric Cholestatic Liver Disease
Hepatol Commun. 2020 Aug 5;4(11):1694-1707. doi: 10.1002/hep4.1574. eCollection 2020 Nov.
Benjamin L Shneider1, Nathan P Goodrich2, Wen Ye3, Cindy Sawyers4, Jean P Molleston4, Robert M Merion23, Daniel H Leung1, Saul J Karpen5, Binita M Kamath6, Laurel Cavallo1, Kasper Wang7, Jeffrey H Teckman8, James E Squires9, Shikha S Sundaram10, Philip Rosenthal11, Rene Romero5, Karen F Murray12, Kathleen M Loomes13, M Kyle Jensen14, Jorge A Bezerra15, Lee M Bass16, Ronald J Sokol10, John C Magee3, Childhood Liver Disease Research Network (ChiLDReN)
Author information
1Baylor College of Medicine and Texas Children's Hospital Houston TX USA.
2Arbor Research Collaborative for Health Ann Arbor MI USA.
3University of Michigan Ann Arbor MI USA.
4Riley Hospital for Children Indiana University Indianapolis IN USA.
5Children's Healthcare of Atlanta and Emory University School of Medicine Atlanta GA USA.
6The Hospital for Sick Children and the University of Toronto Toronto Canada.
7Children's Hospital Los Angeles Toronto Canada.
8School of Medicine Saint Louis University St. Louis MI USA.
9UPMC Children's Hospital of Pittsburgh Pittsburgh PA USA.
10University of Colorado School of Medicine Children's Hospital Colorado Pittsburgh PA USA.
11University of California San Francisco San Francisco CA USA.
12Seattle Children's Hospital and the University of Washington School of Medicine Seattle WA USA.
13The Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania Philadelphia PA USA.
14Primary Children's Hospital and University of Utah Salt Lake City UT USA.
15Cincinnati Children's Hospital Medical Center Cincinnati OH USA.
16Ann and Robert H. Lurie Children's Hospital and Northwestern University Chicago IL USA.
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.