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Kayser-Fleischer Rings
Absent Present
Neurologic Symptoms
Absent Mild Severe
Serum Ceruloplasmin
Normal (>0.2 g/L) 0.1-0.2 g/L < 0.1 g/L
Coombs-negative Hemolytic Anemia
Absent Present
Liver Copper (in the absence of cholestasis)
Normal (< 0.8 umol/g) 0.8-4 umol/g > 5 x ULN (> 4 umol/g) Rhodanine-positive granules
Urinary Copper (in the absence of cholestasis)
Normal 1-2 x ULN > 2 x ULN Normal, but >5 x ULN after d-penicillamine
Mutation Analysis
No Mutations On 1 Chromosome Detected On Both Chromosomes Detected

Wilsons Disease Scoring System (Leipzig Score)

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