Dental considerations in the patient with Wilson's disease.

ABSTRACT

Wilson's disease was described by Wilson in 1912. It is an autosomal recessive disorder caused by mutations in the ATP7B gene, a membrane-bound copper transporting ATPase. The deficiency of ATP7B protein impairs the biliary copper excretion, resulting in positive copper balance, hepatic copper accumulation, and copper toxicity from oxidant damage. The disease is a form of copper poisoning caused by a defect in the transport of copper that renders the patient unable to handle trace amounts of copper normally present in the diet and hence the clinical manifestations are those typically caused by copper toxicity and primarily involve the liver and the brain. Because effective treatment is available, it is important to make an early diagnosis. In this article, a review of clinical aspects of Wilson's disease, and its impact on dental management and dental considerations are discussed.