Distribution of non-ceruloplasmin-bound copper after i.v. ⁶⁴Cu injection studied with PET/CT in patients with Wilson disease.

ABSTRACT:

Background & aims

In Wilson disease (WD), copper accumulation and increased non-ceruloplasmin-bound copper in plasma lead to liver and brain pathology. To better understand the fate of non-ceruloplasmin-bound copper, we used PET/CT to examine the whole-body distribution of intravenously injected 64-copper (⁶⁴Cu).

Methods

Eight patients with WD, five heterozygotes, and nine healthy controls were examined by dynamic PET/CT for 90 min and static PET/CT up to 20 h after injection. We measured ⁶⁴Cu activity in blood and tissue and quantified the kinetics by compartmental analysis.

Results

Initially, a large fraction of injected ⁶⁴Cu was distributed to extrahepatic tissues, especially skeletal muscle. Thus, across groups, extrahepatic tissue

SUBMITTER: Munk DE 

PROVIDER: S-EPMC10597763 | biostudies-literature | 2023 Nov

REPOSITORIES: biostudies-literature