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Serum 'free' copper in Wilson disease
1Department of Medicine, University of Cambridge, Addenbrookes Hospital Cambridge, Cambridge, UK. penicillamine@waitrose.com
Serum "free" copper and urine copper levels in Wilson disease patients show a variable relationship, not a direct linear one. The term "free" copper is inaccurate and should be replaced with "noncaeruloplasmin bound copper".
Area of Science:
- Biochemistry
- Clinical Medicine
- Genetics
Background:
- The relationship between serum "free" copper and urinary copper in Wilson disease is not well-established.
- Wilson disease is a genetic disorder of copper metabolism.
Purpose of the Study:
- To investigate the direct relationship between serum "free" copper and urine copper in Wilson disease patients.
- To analyze copper levels before and after treatment.
Main Methods:
- Retrospective review of 320 Wilson disease patients' case notes (1960-1987).
- Analysis of serum "free" copper and urine copper in 80 treatment-naive patients at admission and one year post-treatment.
Main Results:
- A variable ratio between serum "free" copper and urine copper was observed, averaging 7:1 pre-treatment and 5:1 post-treatment (excluding acute hemolysis cases).
- No direct linear relationship was found between these two parameters, with significant scatter in results.
Conclusions:
- The term "free" copper is imprecise; "noncaeruloplasmin bound copper" is more accurate.
- Most "free" copper is albumin-bound and excreted only with significant renal protein loss.
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