Application of attenuation coefficient in the assessment of hepatic involvement in children and adolescents with Wilson’s disease

Abstract

Background To investigate whether the attenuation coefficient (ATT) can be used as a noninvasive index to assess liver involvement in children and adolescents with Wilson’s disease (WD). Methods Children and adolescents diagnosed with WD were retrospectively collected from the First Affiliated Hospital of the Anhui University of Traditional Chinese Medicine between May 2022 and August 2022. The findings on ATT, Shear Wave Measurement (SWM), AST to platelet ratio index (APRI), and fibrosis 4 (FIB-4) score were obtained. The liver involvement of WD was classified into 3 groups based on serum levels of collagen type IV (CIV), hyaluronic acid (HA), laminin (LN) and precollagen type III N-terminal peptide (PIIINP): (1) Group1 (n = 25), no abnormalities in CIV, HA, LN and PIIINP; (2) Group2 (n = 19), elevation of 1 or 2 indexes in CIV, HA, LN, and PIIINP; Group3 (n = 18), elevation of 3 or 4 indicators in CIV, HA, LN, and PIIINP. The levels of ATT, SWM, APRI and FIB-4 were compared between the 3 groups; and correlation of ATT with SWM and triglyceride (TG) was performed using Spearman's correlation analysis. The Receiver operating characteristic (ROC) curve was used to analyze the diagnostic efficacy of ATT alone and its combination with SWM, APRI, and FIB-4 in children and adolescents with WD. Results A total of 62 children and adolescents with WD were retrospectively retrieved. ATT levels were significantly different in intergroup comparisons (P < 0.001). The ROC curve showed that the area under the curve (AUC) for the diagnosis of hepatic steatosis using ATT was 0.714, 0.712 and 0.867 in Group 1 versus Group 2, Group 2 versus Group 3, and Group 1 versus Group 3, respectively; the sensitivity for the diagnosis of hepatic steatosis in Group 1 versus Group 2 was 89.47% with the cutoff value of ATT of 0.73 dB/cm/MHz. No significant correlation found between ATT and TG (ρ = 0.154, P = 0.231). Compared to ATT alone, the combination of ATT with APRI and FIB-4 or the combination of ATT with SWM, APRI, and FIB-4 showed a better diagnostic efficacy in Group 1 versus Group 2 (both P = 0.038). Conclusion ATT could be used as a non-invasive index for the evaluation of liver steatosis in children and adolescents with WD, with a good clinical applicative value. Furthermore, ATT in combination with APRI, FIB-4, and SWM might have better diagnostic efficacy than ATT alone.

Elastography of the Liver in Wilson’s Disease

“…Group One had normal CIV, HA, LN, and PIIINP; group two had an elevation of one or two markers; whereas group three had an elevation of three markers out of four. In this study, the sensitivity for the diagnosis of hepatic steatosis in Wilson's disease was 89.47% with the cut-off value of ATT of 0.73 dB/cm/MHz, while the combination of ATT with APRI and FIB-4, or the combination of ATT with elastography, APRI, and FIB-4, showed a better diagnostic accuracy [22]. In the same study, elastographically measured liver stiffness was increased in all groups.…”

“…The estimation of the fat volume is performed according to the difference in the degree of attenuation of the signal received by the ultrasound probe. ATT has been developed 20 years ago and since then extensively studied in NAFLD [56]; however, the first study on ATT in Wilson's disease has been published recently [22].…”

“…Recently, a Wang et al study based on a combination of non-invasive methods including elastography and ATT in Wilson's disease has been published. ATT in combination with APRI, FIB-4, and elastographically measured liver stiffness have been proven to be accurate for the diagnosis of hepatic steatosis in patients with Wilson's disease [22]. In a Wang et al study with sixty-two children and adolescents with Wilson's disease, ATT, elastographic liver stiffness, APRI, and FIB-4 were compared between groups of patients categorized for liver involvement by collagen type IV (CIV), hyaluronic acid (HA), laminin (LN), and pre-collagen type III N-terminal peptide (PIIINP) serum concentration.…”