Congenital Wilson's disease is characterized by:
b. May present as acute hepatitis(Ref: Nelson's 20/e p 1939-1940, Ghai 8/e p 320-321)Kayser-Fleischer rings are absent in young patients with hepatic Wilson disease up to 50% of the time but are present in 95% of patients with neurologic symptoms.
Inborn Errors of Metabolism
KF ring is present at birth
May present as acute hepatitis
Decreased urinary copper excretion
Decreased hepatic copper concentration
May present as acute hepatitis