Practical insights into chronic management of hepatic Wilson’s disease

Figure 1 Broad guidance for Wilson’s disease workup interpretation. ¹Only valid if holo-ceruloplasmin is calculated with an enzymatic test. ²Higher probability/³Lower probability of overtreatment or non-compliance. ⁴Non-compliant patients who take DPA on the day of the 24 h urinary collection; UCE in non-compliant patients who do not take DPA > 48 h prior to the assessment should be interpreted as D-penicillamine 48 h cessation test results. ⁵UZE < 2 mg/24 h in non-compliance/UZE >2 mg/24 h in non-response. DPA: D-penicillamine; DPA CT: D-penicillamine 48 h cessation test; NCC: Non-ceruloplamin-bound copper; UCE: Urinary copper excretion; UZE: Urinary zinc excretion.