Therapeutic targets
- Copper detoxification (copper detoxification).
- Balanced copper balance
Therapy recommendations
A distinction is made between initial therapy and maintenance therapy:
- Initial therapy, i.e. treatment with chelating agents (these form complexes with metals; first-choice therapy), zinc salts* – the aim here is to bring the body into a negative copper balance.
- Maintenance therapy i.e. treatment with treatment with chelating agents, zinc salts* – establishment of a balanced copper balance / normal copper homeostasis.
- See also under “Further therapy”.
* Zinc is used mainly in asymptomatic patients and for maintenance therapy.
Therapy must be started as early as possible so as not to limit life expectancy. This is not shortened in individuals who are not yet symptomatic and in individuals with early initiation of treatment for manifest disease.
Therapy must be continued throughout life.
Other treatment options
| Active ingredient group | Active ingredients | Dosage | Special features |
| Antioxidants | Vitamin E | 200-400 IU/d | Adjuvant therapyNo studies |
- Mode of action antioxidants: cytoprotection.
- Side effects: none relevant
Emergency therapy
Fulminant liver failure
- To bridge the waiting period until liver transplantation (LTx), albumin is used to try to lower excessively elevated copper levels; peritoneal dialysis, exchange transfusion, hemofiltration must be performed immediately thereafter
Symptomatic therapy
Symptomatic therapy options are used when symptoms persist for >2 years.
The following groups of agents may be used, among others:
- Antidepressants
- Antipsychotics
- Botulinum toxin
- Clonazepam
- L-dopa
- Tiapride
- Vitamin B6
