Trientine is a medication used to treat a condition called Wilson's disease, a genetic disorder that causes copper to accumulate in various organs of the body, particularly the liver, brain, and eyes. This accumulation can lead to serious health problems if left untreated. Trientine works by binding to excess copper in the body, allowing it to be excreted through urine. It's often used when penicillamine, another medication commonly used for Wilson's disease, cannot be tolerated or is ineffective. Trientine is typically taken orally in the form of capsules or tablets.

Trientine (Syprine) is a chelating agent that forms soluble complexes with copper, iron, and zinc resulting in its excretion via urine. It is used in the treatment of patients with Wilson's disease, especially those who are intolerant to penicillamine.

Trientine Uses:

• Wilson disease:
  • It is indicated for the treatment of patients with Wilson disease who are intolerant to penicillamine.
  • Limitations of use: It is not recommended if the patient has concomitant rheumatoid arthritis and cystinuria. It is also not indicated for the treatment of biliary cirrhosis.

Trientine (Syprine) Dose in Adults

Trientine (Syprine) Dose in the treatment of Wilson disease:

• The usual starting dose is 750 to 1,250 milligrams per day, split into 2 to 4 doses throughout the day.
• If the patient doesn't respond well or if their copper levels remain high, the dose can be increased.
• The maximum daily dose is 2,000 milligrams.
• According to the AASLD practice guidelines, typical doses range from 750 to 1,500 milligrams per day, taken in 2 to 3 doses, with a maintenance dose of 750 to 1,000 milligrams per day.
• Doctors will often adjust the dosage every 6 to 12 months to find the best long-term dose for each patient.

Trientine (Syprine) Dose in Children

Trientine (Syprine) Dose in the treatment of Wilson disease:

Children and Adolescents:

• The initial dose is typically 20 milligrams per kilogram of body weight per day, rounded to the nearest 250 milligrams.
• This dose is divided into 2 to 3 doses throughout the day.
• The maximum initial daily dose is 1,000 milligrams.
• Doctors will adjust the dose based on how the patient responds to treatment and their copper levels in the blood or urine.
• The usual maintenance dose ranges from 900 to 1,500 milligrams per day, also divided into 2 to 3 doses.
• The maximum daily dose is 1,500 milligrams for children and 2,000 milligrams for adolescents.

Pregnancy Risk Category: C

• During pregnancy, it's important to keep treating Wilson's disease, but the dose might need to be reduced by 25% to 50% from what the person was taking before getting pregnant.
• This adjustment is recommended to ensure the safety of both the mother and the baby.

Use during breastfeeding:

• It's uncertain whether trientine passes into breast milk.
• As a precaution, breastfeeding women should use trientine with caution, as its effects on nursing infants are not fully understood.

Dose in Kidney Disease:

• The manufacturer's labeling does not include specific dosage adjustments for individuals with renal impairment.

Dose in Liver disease:

• The manufacturer's labeling does not include specific dosage adjustments for individuals with hepatic impairment.

Side effects of Trientine (Syprine):

• Central Nervous System:
  • Dystonia
  • Myasthenia Gravis
  • Neurological Deterioration.
• Endocrine & Metabolic:
  • Iron Deficiency
• Gastrointestinal:
  • Gastritis.
• Hypersensitivity:
  • Fixed Drug Eruption.
• Neuromuscular & Skeletal:
  • Muscle Spasm
  • Systemic Lupus Erythematosus

Contraindications to Trientine (Syprine):

• If someone has a hypersensitivity (allergic) reaction to trientine or any ingredient in the formulation, they should not take it.
• It's crucial to avoid any medication that could trigger an allergic response, as this could lead to serious complications.

Warnings and precautions

Anemia:

• Trientine can sometimes lead to iron-deficiency anemia, particularly in women.
• It's essential to monitor closely for any signs of anemia, such as fatigue, weakness, or pale skin.

Copper deficiency:

• Treatment with trientine can lead to copper deficiency, which may result in hepatic iron overload or sideroblastic anemia.
• It's crucial to monitor for signs of copper deficiency regularly during treatment.
• If copper levels drop too low, the dose may need to be reassessed.

Neurologic decline:

• When starting treatment with trientine, some individuals may experience neurological worsening, although this is less common compared to treatment with penicillamine.
• It's important to be aware of this possibility and to monitor for any neurological symptoms closely, such as changes in movement or cognition.

Trientine: Drug Interaction

Monitoring parameters:

Monitoring during Trientine Treatment

• Urinary Copper Assessment: Every 6 to 12 months, your doctor may ask for a 24-hour urine collection to check copper levels.
• Serum Non-Ceruloplasmin Bound Copper: This blood test helps monitor copper levels in your body.
• Liver Function Tests (LFTs): These tests evaluate how well your liver is functioning and if there are any signs of liver problems.
• Complete Blood Count (CBC): This test checks for anemia or other blood-related issues.
• INR (International Normalized Ratio): It measures how well your blood clots and is essential if you're on blood thinners.
• Urinalysis: This helps detect any kidney-related problems.
• Early Symptoms: Watch out for fever and changes in your skin during the first month of therapy, and inform your doctor if you notice any.

How to administer Trientine (Syprine)?

Administration Instructions for Trientine

• Timing: Take Trientine at least 1 hour before or 2 hours after meals. Also, ensure there's at least a 1-hour gap between Trientine and any other medication, food, or milk.
• Method: Swallow the capsule whole with water. Do not open or chew it.
• Skin Contact: If any skin comes into contact with the capsule contents, wash the area promptly with water to avoid irritation or adverse reactions.

Mechanism of action of Trientine (Syprine):

• Trientine is an oral chelating agent that's different from penicillamine and other similar drugs.
• It's effective at removing excess copper from the body by promoting the excretion of copper through urine, a process known as cupriuresis.

Absorption:

• Trientine has poor absorption from the digestive system.

Metabolism:

• It's metabolized into acetyltrien, which has much weaker chelating activity compared to the parent compound.

Excretion:

• Trientine and its metabolites are primarily excreted through urine, with only a small portion (1% as the parent compound and 8% as the metabolite) being eliminated in this way.

International Brand Names of Trientine:

• Syprine
• Cuprior

Trientine Brand Names in Pakistan:

No Brands Available in Pakistan.