Non-invasive evaluation of hepatic manifestation in Wilson disease with transient elastography, ARFI, and different fibrosis scores

Abstract

TE displayed a gradual increase between different stages of hepatic manifestation in WD and could significantly discriminate cirrhosis. The TE cutoff for cirrhosis may be clinically more relevant than the R-ARFI value.

Transient elastography of the liver in children with Wilson's disease: Preliminary results

“…In contrast, Sini et al, 25 in a study of 35 WD patients, identified an 8.4-kPa cutoff for the discrimination of severe fibrosis, and Karlas et al 14 found an even lower cutoff, 6.1 kPa, for the identification of cirrhosis. These lower cutoffs for severe and significant fibrosis than those reported for other etiologies 3,17 stress the fact that elastographic values may depend on the cause of the liver disease.…”

“…14, 24 Karlas et al, 14 citing a study published in 1980, 27 suggest focal hepatic accumulation as an etiology-specific feature that may influence LS in WD; furthermore, they hypothesized that these differences between WD patients and groups of patients with other etiologies may be the result of several study-specific characteristics and diseasespecific properties, such as clinical scoring systems being used to classify the degree of hepatic involvement rather than histologic criteria. 1,2,14 They also mentioned that the patients in their study group had already been undergoing longterm treatment for WD and most had had a stable disease course after the initial improvement.…”

“…Curves illustrate (A) the individual changes in nine patients (C1-9) and (B) the overall tendency of liver stiffness to decrease in the entire group from baseline through follow-up intervals 1 and 2 (T1 and T2). Only a few studies have attempted to use TE to assess fibrosis in patients with WD, 14,25,26 probably because the relative rarity of WD may render difficult the study of large cohorts of patients elsewhere than in specialized centers. 14 In addition, it has been proven and generally accepted that not only extracellular matrix accumulation (fibrosis) leads to increments in LS: many other conditions (intrahepatic deposits among them) may also influence LS measurements.…”

“…Only a few studies have attempted to use TE to assess fibrosis in patients with WD, 14,25,26 probably because the relative rarity of WD may render difficult the study of large cohorts of patients elsewhere than in specialized centers. 14 In addition, it has been proven and generally accepted that not only extracellular matrix accumulation (fibrosis) leads to increments in LS: many other conditions (intrahepatic deposits among them) may also influence LS measurements. 15 On the basis of this concept, we therefore hypothesized that intrahepatic copper accumulation in WD patients may lead to increased LS and, conversely, liver copper excretion may decrease LS values.…”

“…13 We hypothesized that copper accumulation in the liver can similarly lead to an increase in LS. 14,15 During specific chelation therapy, LS may decrease. Therefore, the aim of this study was to investigate the changes in LS measurements during treatment in pediatric patients with WD.…”