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

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.

Update Alpha-1-Antitrypsin-Mangel

“…Dabei istbesonders auf sonographischeIndikatoren einer sich entwickelnden portalen Hypertension zu achten (zunehmende Milzgröße, Rarefizierung intrahepatischer Portaläste, Reduktion oder gar Umkehr des portalen Blutflusses in der Dopplersonographie). Die Leberelastographie kann ergänzend hilfreich sein [24,25]. Spezifische sonographische Veränderungen, die auf einen Alpha-1-Antitrypsin-Mangel hinweisen, gibt es allerdings nicht.…”

“…B. Gerinnung, Albumin, Cholinesterase oder die Serumspiegel fettlöslicher Vitamine zu empfehlen. Neben der Sonographie zur Beurteilung des Leberparenchyms und der Dokumentation der Milzgröße sollte, wenn möglich, auch eine Leberelastographie erfolgen [25]. Bei Zunahme der Fibrose ist eine Intensivierung der Betreuung anzustreben.…”

Application of Ultrasound Elastography in Assessing Portal Hypertension

“…The study indicated a positive correlation between several biochemical indicators (total bilirubin, ALT, AST, gamma-glutamyltranspeptidase (GGT), international normalized ratio (INR), GGT to platelet ratio (GPR), AST to platelet ratio, spleen size, pediatric end-stage liver disease score, and the existence of portal hypertension. Meanwhile, the study also pointed out a more detailed correlation of LSM and portal hypertension grades, indicating the important role of LSM in classifying both the existence and degree of portal hypertension [ 23 ]. Another study published in 2020 included 140 patients with clinically significant portal hypertension (CSPH) and investigated the diagnostic performance of LSM of portal hypertension.…”

Alagille Syndrome: A Focused Review on Clinical Features, Genetics, and Treatment

“…Use of FibroScan for this purpose in children with ALGS remains investigational and is not yet part of routine clinical care. 90…”