Cuvrior (trientine) is a copper chelating agent, with trientine tetrahydrochloride as its main active ingredient. It is indicated for the treatment of Wilson's disease in adult patients who have achieved copper depletion and are tolerant to penicillamine.

How Effective is Cuvrior (Trientine) in Treatment?

Regulation of Copper Metabolism

As a copper chelator, it forms stable complexes with excess copper in the body, facilitating the excretion of copper through urine.

It can reduce intestinal absorption of copper, achieving bidirectional regulation of copper levels.

Clinical studies have shown that this medication effectively maintains stable levels of serum non-ceruloplasmin copper (NCC) in patients.

Long-Term Disease Control

For patients who have undergone copper-depletion therapy and are tolerant to penicillamine, switching to trientine can maintain clinical stability.

Its therapeutic efficacy is comprehensively evaluated based on 24-hour urinary copper excretion (UCE) and serum NCC levels.

Efficacy Characteristics

Common adverse reactions include abdominal pain, changes in bowel habits, and skin rashes, most of which are mild to moderate in severity.

Alertness is required for potential worsening of neurological symptoms (associated with copper mobilization) during the initial treatment phase and copper/iron deficiency caused by long-term medication use.

Target Population for Cuvrior (Trientine)

Indicated Patients

Adult patients with Wilson's disease who have completed copper-depletion therapy and are tolerant to penicillamine.

Patients requiring long-term maintenance treatment.

Contraindicated Population

Patients allergic to trientine or any excipients in Cuvrior.

Patients with severe hepatic or renal impairment (dose adjustment is required with caution).

Patients with cardiovascular diseases who are currently using nitrates.

Medication Monitoring for Cuvrior (Trientine)

Pre-Treatment Assessment

Baseline measurements of serum NCC and 24-hour UCE.

Assessment of hepatic and renal function, as well as risks of drug-drug interactions.

During-Treatment Monitoring

Laboratory Monitoring:

Recheck NCC 3 months after the start of treatment, followed by regular monitoring every 6 months thereafter.

Measure UCE every 6-12 months.

Clinical Assessment: Monitor for neurological symptoms, skin reactions, and signs of copper/iron deficiency.

Dose Adjustment: Dynamically adjust the dose based on monitoring results, with a maximum daily dose not exceeding 3000mg.

Management of Emergency Situations

Seek immediate medical attention if priapism (persistent erection lasting >4 hours) or severe allergic reactions occur.

For symptoms of copper deficiency (e.g., anemia), copper supplementation should be administered and the treatment regimen adjusted accordingly.