Dhawan A et al. (2005) Wilson's disease in children: 37-year experience and revised King's score for liver transplantation. Liver Transpl 11: 441–448

A team from King's College Hospital in the UK have recently reported on their 37-year experience of treating children with Wilson's disease, and have proposed a new method for predicting mortality in these patients. This rare disorder of copper metabolism can often be treated successfully with chelating agents, but liver transplantation is sometimes necessary. It is important, therefore, to assess the likelihood of survival without transplantation.

Dhawan et al. first described their set of prognostic criteria for predicting death without transplantation in 1986. This retrospective review of 74 cases of Wilson's disease showed that the original scoring system—based on bilirubin, aspartate aminotransferase and INR—had a sensitivity of 87% and a specificity of 90%. The team then refined the system, adding albumin and white cell count to the list of criteria. The new Wilson Index identified a cut-off score of 11 for death without transplantation. Using the revised parameters, sensitivity and specificity were improved to 93% and 98%, respectively.

Next, the investigators tested the new index prospectively in a further 14 patients. All four children in whom transplantation was indicated had a Wilson Index score of at least 11, although one child had a score of 11 and did not require a transplant. All of the children who responded to medical treatment had a score of 6 or lower.

In conclusion, the new Wilson Index appears to be superior to the previous scoring system in predicting mortality in children with Wilson's disease. A larger group of patients is now needed to validate these findings.