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Insufficient evidence to make a recommendation.

Strongly favor this test or intervention.

The Ferenci scoring system evaluates the likelihood of Wilson's Disease (WD) using specific clinical and genetic indicators. Each input corresponds to a particular sign or symptom associated with Wilson's Disease, and each option carries a specific score. This score helps determine the probability of the disease being present.

Here's how the scoring is structured:

- Kayser-Fleischer rings: 
  - Absent = 0
  - Present = 2

- Neuropsychiatric symptoms suggestive of Wilson's Disease:
  - Absent = 0
  - Present = 2

- Coombs-negative hemolytic anemia:
  - Absent = 0
  - Present = 1

- Urinary copper (in the absence of acute hepatitis):
  - Normal = 0
  - 1-2x upper limit of normal = 1
  - >2x upper limit of normal = 2
  - Normal, but >5x upper limit of normal after challenge with 2 x 0.5 g D-penicillamine = 2

- Liver copper quantitative:
  - Normal = 1
  - Up to 5x upper limit of normal = 1
  - >5x upper limit of normal = 2

- Rhodanine-positive hepatocytes (only if quantitative copper measurement is not available):
  - Absent = 0
  - Present = 1

- Serum ceruloplasmin (normal: >20 mg/dL):
  - Normal = 0
  - 10-20 mg/dL = 1
  - <10 mg/dL = 2

- Mutation analysis:
  - Disease-causing mutations on both chromosomes = 4
  - Disease-causing mutations on one chromosome = 1
  - No disease-causing mutation detected = 0

After collecting the responses and converting them into scores, you sum these scores to get the total. This total score then leads to an interpretation about the likelihood of Wilson's Disease:

- A score of 4 or more suggests that a diagnosis of WD is highly likely.
- A score of 2 or 3 indicates that WD is probable.
- A score of 1 or less suggests that WD is unlikely.

This scoring system integrates both clinical symptoms and genetic findings to provide a comprehensive assessment of the risk of Wilson's Disease. The calculated score and its interpretation guide clinicians in their diagnostic considerations.

The Ferenci scoring system is a clinical calculator specifically designed for the diagnosis of Wilson's disease, a rare inherited disorder that causes copper to accumulate in the liver, brain, and other vital organs. This scoring system is based on a combination of clinical, biochemical, and genetic parameters, including the presence of K-F rings, neurological symptoms, serum ceruloplasmin levels, hepatic copper content, and ATP7B mutation analysis.

The Ferenci scoring system is used to establish a diagnosis of Wilson's disease in patients presenting with liver disease, neurological symptoms, or psychiatric disturbances, especially when these symptoms are unexplained or when there is a family history of Wilson's disease. It is particularly useful in cases where the diagnosis is uncertain based on initial testing.

The scoring system is not applicable to asymptomatic patients or those without any clinical or biochemical evidence suggestive of Wilson's disease. It should also not be used in isolation but in conjunction with other diagnostic tests and clinical judgment.

Ferenci scoring system for Wilson's disease

The Revised King's score for liver transplantation in Wilson's disease is a calculator designed to evaluate the need for liver transplantation based on five clinical parameters drawn from lab tests: bilirubin levels, international normalized ratio (INR), aspartate aminotransferase (AST), white cell count, and albumin levels.

The assessment involves receiving input in the form of categorical choices for each parameter. Each choice corresponds to a specific score, and these scores are summed to yield a total score. Here is how scoring is done:

1. Bilirubin, mcol/L:
   - 0-100: 0 points
   - 101-150: 1 point
   - 151-200: 2 points
   - 201-300: 3 points
   - > 301: 4 points

2. International normalized ratio:
   - 0-1.29: 0 points
   - 1.3-1.6: 1 point
   - 1.7-1.9: 2 points
   - 2.0-2.4: 3 points
   - > 2.5: 4 points

3. Aspartate aminotransferase, IU/L:
   - 0-100: 0 points
   - 101-150: 1 point
   - 151-300: 2 points
   - 301-400: 3 points
   - > 401: 4 points

4. White cell count, 10^9/L:
   - 0-6.7: 0 points
   - 6.8-8.3: 1 point
   - 8.4-10.3: 2 points
   - 10.4-15.3: 3 points
   - > 15.4: 4 points

5. Albumin, g/L:
   - > 45: 0 points
   - 34-44: 1 point
   - 25-33: 2 points
   - 21-24: 3 points
   - < 20: 4 points

The total score can range from 0 to 20. The interpretation of the total score is as follows:

- Scores between 0 and 10 predict a successful response to chelation therapy, suggesting that liver transplantation is not immediately necessary.
- Scores from 11 to 20 indicate a need for liver transplantation.

The Revised King's Score is a clinical calculator used specifically for patients with Wilson's disease, a rare inherited disorder that causes copper to accumulate in the liver, brain, and other vital organs. This scoring system is used to determine the necessity and urgency of liver transplantation in these patients. 

The Revised King's Score takes into account various clinical parameters such as bilirubin, albumin, prothrombin time, and presence of neurological symptoms. The higher the score, the greater the need for urgent liver transplantation. 

This calculator is not applicable to patients without Wilson's disease or those with Wilson's disease who do not have liver involvement. It is also not suitable for patients with other causes of liver disease or those who have contraindications to liver transplantation.

Wilson's disease

Guidelines

Key sources