Abstract:Objective To analyze the clinical characteristics of Wilson disease (WD) patients with normal serum ceruloplasmin (CP) level. Methods With serum CP ≥ 200 mg/L as normal value, 82 WD patients with normal serum CP level (normal CP group) admitted from Jan. 2018 to Oct. 2022 were selected as the subjects, and 82 WD patients with serum CP< 200 mg/L admitted at the same time were selected as control group. General data, complications, laboratory parameters, King score, and the copper biochemical indicators before and after treatment were compared between the 2 groups. Results The age of the normal CP group was older than that of the control group (P<0.001). The proportion of hepatic type patients was significantly higher than that of the control group (P<0.001). The incidences of liver cirrhosis and ascites were significantly higher than those of the control group (both P<0.05). Total bile acid, alanine transaminase, aspartate transaminase, γ-glutamyltransferase, ammonia, D-dimer, serum CP, serum copper, copper oxidase, liver fibrosis indexes (hyaluronic acid, laminin, and collagenⅣ), and King score were significantly higher than those of the control group, and albumin was significantly lower than that of the control group (all P<0.05). After treatment, the levels of serum CP, serum copper, and copper oxidase were significantly lower than those before treatment in 58 rehospitalized cases in the normal CP group (all P<0.001), the levels of serum CP in 44.83% (26/58) patients in normal CP group were still ≥ 200 mg/L, and only serum copper level of 66 rehospitalized cases in the control group was significantly lower than that before treatment (P=0.001). Conclusion WD patients with normal serum CP level are mainly hepatic type, with older age, and some patients have more severe liver damage. Serum CP level can be slightly decreased after treatment.