Abstract: Objective The purpose of this study was to observe the shear wave velocity (SWV) by acoustic radiation force impulse (ARFI) in patients with hepatolenticular degeneration (HLD).Methods 38 patients with HLD, 49 with other chronic liver disease (CLD) and 56 healthy persons were recruited in thisstudy between June 2017and June 2019. The ARFI technology was used to measure hepatic SWV. Serum copper ion (Cu²⁺) level was determined by atomic absorption spectrometry, serum ceruloplasmin (CP) level was determined by immunoturbidimetric method, and serum copper oxidase (sCO) were determined by manual method.Serum type III procollagen (PC-III), laminin (LN), type IV collagen (IV-C), and hyaluronic acid (HA) wereassayed by radioimmunoassay.Results Serum Cu^2＋and CP levels in patients with HLD were (6.7±1.5)μmol/L and (88.0±13.2)mg/L, significantly lower than 【(13.2±1.6)μmol/L and (216.6±43.5)mg/L, respectively, P＜0.05】 in patients with CLD, or 【(17.2±6.2)μmol/L and (400.0±150.0)mg/L, respectively, P＜0.05】 in healthy persons, and sCO activity in patients with HLD was (0.02±0.01)U/L, much lower than 【(0.4±0.1)U/L, P＜0.05】 in healthy persons; serum PC-Ⅲ, Ⅳ-C, LN and HA levels in patients with HLD were (11.1±0.3)ng/mL, (95.9±12.4)ng/mL, (43.6±11.4)ng/mL and (88.9±15.8)ng/mL, all significantly lower than 【(48.5±24.2)ng/mL, (140.6±15.3)ng/mL, (847.6±38.5)ng/mL and (261.2±22.8)ng/mL, P＜0.05】 in patients with CLD; the sonogram showed that the liver were fatty liver-like, starlight, dendritic, terraneous or nodular in patients with HLD, and the SWV in patients with HLD was (1.9±0.2)m/s, significantly lower than 【( 2.2±0.3 )m/s,P＜0.05】 in patients with CLD, but much higher than 【(1.1±0.2)m/s, P＜0.05】 in healthy persons.Conclusion The sonogram shows characteristic hepatic changes in patients with HLD, and the SWV might help discriminate with other CLD to some extent.

Key words: Hepatolenticular degeneration, Acoustic radiation force impulse, Shear wave velocity