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.
KF ring is present at birth
May present as acute hepatitis
Decreased urinary copper excretion
Decreased hepatic copper concentration
Inborn Errors of Metabolism