TY - JOUR
T1 - Nonfasted Liver Stiffness Correlates with Liver Disease Parameters and Portal Hypertension in Pediatric Cholestatic Liver Disease
AU - for the Childhood Liver Disease Research Network (ChiLDReN)
AU - Shneider, Benjamin L.
AU - Goodrich, Nathan P.
AU - Ye, Wen
AU - Sawyers, Cindy
AU - Molleston, Jean P.
AU - Merion, Robert M.
AU - Leung, Daniel H.
AU - Karpen, Saul J.
AU - Kamath, Binita M.
AU - Cavallo, Laurel
AU - Wang, Kasper
AU - Teckman, Jeffrey H.
AU - Squires, James E.
AU - Sundaram, Shikha S.
AU - Rosenthal, Philip
AU - Romero, Rene
AU - Murray, Karen F.
AU - Loomes, Kathleen M.
AU - Jensen, M. Kyle
AU - Bezerra, Jorge A.
AU - Bass, Lee M.
AU - Sokol, Ronald J.
AU - Magee, John C.
N1 - Funding Information:
In the last decade, transient elastography (TE) has significantly enhanced clinical monitoring of adults with chronic liver disease, changing the role of and need for liver biopsy. This noninvasive technique, which measures liver stiffness, is particularly useful in differentiating advanced fibrosis on liver biopsy from no or minimal fibrosis in adults.(1) TE is not as well studied in pediatrics, although experience is growing. A meta-analysis of transient and shear wave elastography reports in children with chronic liver disease published before 2017 revealed sensitivity 90%, specificity 79%, and receiver operating characteristic 0.92 for varying definitions of portal hypertension.(2) One of these studies assessed 249 children with cystic fibrosis, while the pediatric studies in biliary atresia (BA) were single center, including up to 73 children.
Publisher Copyright:
© 2020 The Authors. Hepatology Communications published by Wiley Periodicals LLC on behalf of the American Association for the Study of Liver Diseases.
PY - 2020/11/1
Y1 - 2020/11/1
N2 - 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.
AB - 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.
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U2 - 10.1002/hep4.1574
DO - 10.1002/hep4.1574
M3 - Article
C2 - 33163838
AN - SCOPUS:85102384807
SN - 2471-254X
VL - 4
SP - 1694
EP - 1707
JO - Hepatology Communications
JF - Hepatology Communications
IS - 11
ER -