Current Drug Managements of Wilson's Disease: From West to East

Curr Neuropharmacol. 2016;14(4):322-5. doi: 10.2174/1570159x14666151130222427.

Abstract

Wilson's disease (WD), also called hepatolenticular degeneration, is an autosomal recessive inheritance disorder of copper metabolism characterized by the multiple mutations in the ATP-ase 7B gene of chromosome 13q. About half of the WD patients have neurological or psychiatric symptoms. As WD is a kind of medicable or nearly curable neurodegenerative disease in the field of medicine, early consideration/examination and without delay/ life-long treatment usually lead to better prognoses. The drugs, also named as anticopper agents, are commonly used in clinics including D-penicillamine, trientine, sodium dimercaptosuccinate, dimercaptosuccinic acid, zinc and tetrathiomolybdate. This provides detailed reviews about these medicines.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Chelating Agents / therapeutic use*
  • Copper / metabolism
  • Hepatolenticular Degeneration / drug therapy*
  • Hepatolenticular Degeneration / metabolism
  • Humans
  • Medicine, Chinese Traditional
  • Molybdenum / therapeutic use
  • Penicillamine / therapeutic use
  • Succimer / therapeutic use
  • Treatment Outcome
  • Trientine / therapeutic use
  • Zinc / therapeutic use

Substances

  • Chelating Agents
  • Copper
  • Molybdenum
  • tetrathiomolybdate
  • Succimer
  • Penicillamine
  • Zinc
  • Trientine