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• W2892260985 abstract "Copper toxicity is strongly linked to its free fraction also called non-ceruloplasmin-bound copper. The assay of exchangeable copper (CuEXC), a new method of direct determination of so-called free copper, is reliable, rapid, and easy to set up. The relative CuEXC (REC) (ratio CuEXC/total serum copper) was studied in three different populations [Wilson disease (WD), ATP7B simple heterozygotes and normal subjects] and compared to the usual copper tests. A REC value >18.5% appears as an excellent biomarker for the diagnosis of WD offering 100% sensitivity and specificity, regardless of sex, age, or the degree of underlying liver failure. Moreover, another study reported that REC was always <18.5% in numerous non-Wilsonian chronic or acute liver diseases. In family screening, REC determination also discriminates significantly subjects with or without WD with a cutoff of 15%. Another strength of this biomarker is that CuEXC value is closely linked to extrahepatic lesions: a value over 2.08 µmol/L determining corneal or/and cerebral involvement. Furthermore, CuEXC at diagnosis reflects the severity of the extrahepatic disease, as it positively correlates with the neurological symptoms and disability, the severity of corneal copper deposits, and the dissemination of MRI cerebral lesions." @default.
• W2892260985 created "2018-09-27" @default.
• W2892260985 creator A5002664988 @default.
• W2892260985 creator A5043568488 @default.
• W2892260985 date "2019-01-01" @default.
• W2892260985 modified "2023-10-14" @default.
• W2892260985 title "Direct Determination of Non-Ceruloplasmin-Bound Copper in Plasma" @default.
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• W2892260985 doi "https://doi.org/10.1016/b978-0-12-810532-0.00022-7" @default.
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