Wilson’s Disease Scoring System

Kayser-Fleischer Rings :	AbsentPresent
Neurologic Symptoms :	AbsentMildSevere
Serum Ceruloplasmin :	Normal (>0.2 g/L)0.1-0.2 g/L< 0.1 g/L
Coombs-negative Hemolytic Anemia :	AbsentPresent
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) :	Normal1-2 x ULN> 2 x ULNNormal, but >5 x ULN after d-penicillamine
Mutation Analysis :	No MutationsOn 1 Chromosome DetectedOn Both Chromosomes Detected

Formula :

Points are given for each variable.

Explanation of Result :

4 or more: Diagnosis established
3: Diagnosis possible, more tests needed
2 or less: Diagnosis very unlikely

* Please verify all calculations prior to clinical use.