Wilson’s disease (WD) diagnosis and therapy monitoring benefit from knowing the 24-hour urinary copper excretion (UCE) levels. Exchangeable copper (ExC) is a brand-new potential marker, although chelation treatment patients have never had their long-term modifications reported. For a study, researchers sought to outline the long-term changes in ExC levels relative to UCE levels in pediatric patients with symptomatic WD receiving chelation treatment.

At the National Reference Center for WD in Lyon, a retrospective, descriptive, and analytical investigation was carried out on all patients under the age of 18, diagnosed between 2006 and 2020, and receiving chelation treatment. At the time of diagnosis and follow-up, the levels of ceruloplasmin, serum copper, 24 h-UCE, ExC, and liver enzymes were examined.

About 36 participants in the study, 31 of whom had the hepatic type of WD (n = 31), were included. The median [interquartile range (IQR)]  age at diagnosis was 10.5 years (range: 8.4–13.1), and the median (IQR) follow-up time was 6.3 years (range: 3.3–8.8). The ExC value at diagnosis was 1.01 (0.60-1.52) µmol/L, the median (IQR) value. There was stability after a considerable decline over the first year of chelation therapy (P=0.0008). ExC levels ranged from 0.38 (0.22-0.63) µmol/L at 12 to 18 months to 0.43 (0.31-0.54) µmol/L at 5 years (P=0.4057), with the median (IQR) values being 0.38 (0.22-0.63) µmol/L at both time points. Similar to this, 24-hour UCE had a considerable decline during the first year of chelation therapy (P<0.001), followed by stability.

The dynamics of both biomarkers were similar over the course of the follow-up, indicating their use in clinical practice for monitoring WD. The investigation revealed a considerable decline in ExC and 24-hour UCE levels over the first year of follow-up.

Reference: journals.lww.com/jpgn/Abstract/2022/10000/Long_Term_Urinary_Copper_Excretion_and.22.aspx