In
a neurological setting, diagnosis of WD is easier, as a
KF ring would be positive in almost all cases and along
with either a low ceruloplasmin or high urinary copper,
would be diagnostic. In liver disease, diagnosis can be
more complex. WD is strongly suggested by any two of
the following – low ceruloplasmin, high urinary copper,
presence of KF rings, and confirmed by a high hepatic
Cu. If a liver biopsy is not possible due to coagulopathy,
but other investigations are suggestive of WD, chelation
therapy can be started immediately. Liver biopsy must
then be done at the earliest opportunity, as hepatic copper may remain
elevated despite years of therapy and clin-
ical improvement
Diagnostic
approach