title: 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.
authors.affiliation: Department of Oral Medicine and Radiology, Sharad Pawar Dental College and Hospital, DMIMS (DU), Sawangi (M), Wardha, Maharashtra, India. dr21lohe@rediffmail.com
authors.forename: Vidya K
authors.initials: VK
authors.lastname: Lohe
authors.forename: Ravindra P
authors.initials: RP
authors.lastname: Kadu
authors.forename: Shirish S
authors.initials: SS
authors.lastname: Degwekar
authors.forename: Rahul R
authors.initials: RR
authors.lastname: Bhowate
authors.forename: Anil K
authors.initials: AK
authors.lastname: Wanjari
authors.forename: Suwarna B
authors.initials: SB
authors.lastname: Dangore
mesh_terms: D000251:Adenosine Triphosphatases / Q000172:deficiency / Q000235:genetics; D000978:Antiparkinson Agents / Q000009:adverse effects; D001037:Aphasia / Q000209:etiology; D027682:Cation Transport Proteins / Q000172:deficiency / Q000235:genetics; D002614:Chelating Agents / Q000627:therapeutic use; D000075202:Contraindications; D000073840:Copper-Transporting ATPases; D017742:Dental Care for Chronically Ill; D003731:Dental Caries / Q000209:etiology; D003937:Diagnosis, Differential; D004347:Drug Interactions; D006527:Hepatolenticular Degeneration / Q000150:complications* / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D006801:Humans; D009069:Movement Disorders / Q000209:etiology; D009969:Orthodontic Wires; D012798:Sialorrhea / Q000209:etiology; D014987:Xerostomia / Q000139:chemically induced
chemical_list: D000978:Antiparkinson Agents; D027682:Cation Transport Proteins; D002614:Chelating Agents; D000251:Adenosine Triphosphatases; C000612464:ATP7B protein, human; D000073840:Copper-Transporting ATPases