Non-ceruloplasmin copper and urinary copper in clinically stable Wilson disease: Alignment with recommended targets.

ABSTRACT:

Background & aims

Wilson disease (WD) is caused by accumulation of copper primarily in the liver and brain. During maintenance therapy of WD with D-penicillamine, current guidelines recommend on-treatment ranges of urinary copper excretion (UCE) of 200-500 μg/24 h and serum non-ceruloplasmin-bound copper (NCC) of 50-150 μg/L. We compared NCC (measured by two novel assays) and UCE from patients with clinically stable WD on D-penicillamine therapy with these recommendations.

Methods

This is a secondary analysis of data from the Chelate trial (NCT03539952) that enrolled physician-selected patients with clinically stable WD on D-penicillamine maintenance therapy (at an unaltered dose for at least 4 months). We analyzed laboratory samples from the first screening visit, prior to

SUBMITTER: Ott P 

PROVIDER: S-EPMC11321293 | biostudies-literature | 2024 Aug

REPOSITORIES: biostudies-literature