Diagnostic scoring system for Wilson disease.
The Leipzig Score (Wilson Disease) is a GI & Liver scoring tool used in paediatric practice. Diagnostic scoring system for Wilson disease. It scores 8 criteria and interprets the total across 3 bands, so the number always arrives with the severity it implies rather than on its own. In PediAid each criterion is shown as a labelled option with the weight it carries, so the total can be checked rather than taken on trust.
8. They are Kayser-Fleischer rings; Neurological symptoms or typical MRI brain changes; Serum caeruloplasmin; Coombs-negative haemolytic anaemia; Liver copper (in the absence of cholestasis); Rhodanine-positive granules (if copper unavailable); and others.
The total is interpreted as: -1 points or more - Unlikely: score 0-1 - Wilson disease unlikely. Consider alternative causes of liver disease; 2 points or more - Possible: score 2-3 - possible Wilson disease; more investigations needed (24-h urinary copper, penicillamine challenge, liver biopsy, ATP7B sequencing); 4 points or more - Highly likely: score ≥4 - diagnosis of Wilson disease established. Start chelation/zinc therapy, screen siblings, and refer to hepatology.
No. It is a structured way to record and communicate severity. A clinician concerned about a child should act on that concern regardless of the total.
Agreed at the 8th International Meeting on Wilson Disease, Leipzig 2001.
Yes. Score definitions are bundled with the PediAid app, so the score computes without a connection.