| Typical clinical symptoms and signs | Other tests | ||
| KF rings | Liver copper (in the absence of cholestasis) | ||
| Present | 2 | >5x ULN (>4 micromol/g) | 2 |
| Absent | 0 | 0.8-4 micromol/g | 1 |
| Neurologic symptoms* | Normal (<0.8 micromol/g) | –1 | |
| Severe | 2 | Rhodanine-positive granules¶ | 1 |
| Mild | 1 | Urinary copper (in the absence of acute hepatitis) | |
| Absent | 0 | Normal | 0 |
| Serum ceruloplasmin | 1-2x ULN | 1 | |
| Normal (>0.2 g/L) | 0 | >2x ULN | 2 |
| 0.1-0.2 g/L | 1 | Normal, but >5x ULN after D-penicillamine | 2 |
| <0.1 g/L | 2 | Mutation analysis | |
| Coombs-negative hemolytic anemia | On both chromosomes detected | 4 | |
| Present | 1 | On one chromosome detected | 1 |
| Absent | 0 | No mutations detected | 0 |
| TOTAL SCORE | Evaluation: | ||
| 4 or more | Diagnosis established | ||
| 3 | Diagnosis possible, more tests needed | ||
| 2 or less | Diagnosis very unlikely | ||
