Abstract: Objective The aim of this study was to investigate prediction of liver cirrhosis (LC) by shear wave elastography (SWE) and liver fibrosis index in patients with hepatolenticular degeneration (HLD). Methods 52 patients with HLD were encountered in our hospital between December 2022 and December 2024, and all underwent liver biopsies and SWE for Young’s modulus. Aspartate aminotransferase-to-platelet ratio index (APRI) and fibrosis-4 index (Fib-4) were calculated. Serum hyaluronic acid (HA), laminin (LN), type III procollagen amino-terminal peptide (PIIINP) and type IV collagen (IV-C) levels were analyzed. Receiver operating characteristic (ROC) curves were constructed to evaluate the predictive efficacy. Results APRI and FIB-4 scores in 9 patients with LC were (1.1±0.4) and (2.1±0.3), both significantly greater than [(0.5±0.2) and (1.2±0.2), respectively, P<0.05] in 43 patients with HLD; serum HA level was (313.4±54.7)ng/mL, and Young’s modulus was (14.2±2.1)kPa, both much higher or greater than [(142.2±25.4)ng/mL and (7.9±0.9)kPa, respectively, P<0.05] in those with HLD; ROC analysis showed that the AUC was 0.983(95%CI:0.955-1.000), with sensitivity of 97.4% and specificity of 98.1%, when combination of Young’s modulus with APRI or FIB-4 score and serum HA level in predicting LC in patients with HLD. Conclusion Shear wave elastography with combination of serum fibrosis markers or fibrosis index really have an excellent predictive performance in assessing LC in patients with HLD.

Key words: Hepatolenticular degeneration, Shear wave elastography, Aspartate aminotransferase-to-platelet ratio index, Fibrosis-4 index, Diagnosis