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Hepatolenticular Degeneration (Wilson's Disease) Summary

Description: A rare autosomal recessive disease characterized by the deposition of copper in the BRAIN; LIVER; CORNEA; and other organs. It is caused by defects in the ATP7B gene encoding copper-transporting ATPase 2 (EC 3.6.3.4), also known as the Wilson disease protein. The overload of copper inevitably leads to progressive liver and neurological dysfunction such as LIVER CIRRHOSIS; TREMOR; ATAXIA and intellectual deterioration. Hepatic dysfunction may precede neurologic dysfunction by several years.

Also Known As: Wilson's Disease; Wilson Disease; Hepatic Form of Wilson Disease; Hepato-Neurologic Wilson Disease; Hepatocerebral Degeneration Show All >>

Networked: 2730 relevant articles (97 outcomes, 226 trials/studies) for this Disease

Key Drugs and Agents for Hepatolenticular Degeneration

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. Zinc : 32 outcomes 22 studies in 182 results : IBA
  2. Copper : 28 outcomes 105 studies in 1476 results : IBA
  3. Penicillamine (Cuprimine) : 25 outcomes 24 studies in 401 results : FDA 2
  4. tetrathiomolybdate (ammonium tetrathiomolybdate) : 6 outcomes 6 studies in 54 results : IBA
  5. Trientine (Trien) : 4 outcomes 6 studies in 83 results : FDA 1
  6. Zinc Acetate : 4 outcomes 1 study in 14 results : FDA 2
  7. Ceruloplasmin (Ferroxidase) : 3 outcomes 23 studies in 250 results : IBA
  8. Chelating Agents : 3 outcomes 4 studies in 36 results : IBA
  9. Iron : 2 outcomes 9 studies in 75 results : IBA
  10. Salts : 2 outcomes 1 study in 7 results : IBA
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Diseases Related to Hepatolenticular Degeneration

  1. Liver Diseases (Liver Disease)
  2. Cystinuria
  3. Rheumatoid Arthritis
  4. Hepatolenticular Degeneration (Wilson's Disease)
  5. Hemolytic Anemia
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Key Therapies for Hepatolenticular Degeneration

Efficacy Chart >>
  1. Liver Transplantation : 12 outcomes 7 studies in 198 results
  2. Transplantation (Transplant Recipients) : 4 outcomes 3 studies in 48 results
  3. Chelation Therapy (Therapy, Chelation) : 3 outcomes in 21 results
  4. Tissue Therapy (Cell Therapy) : 2 outcomes 1 study in 5 results
  5. Plasma Exchange : 2 outcomes in 13 results
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