Preclinical Studies TN chelates copper by forming a stable complex with four constituent nitrogens and is excreted mainly in the urine. The most common ADRs during TN treatment are sideroblastic anemia (due to TN chelation of iron), lupus-like reactions, hemorrhagic gastritis, loss of taste, and skin changes (rashes). The results are presented in Table 5. Do not chew, break, or open a trientine capsule. Indications and dose Show For trientine Show For Cufence Show For Cuprior Interactions View interactions for trientine Side-effects For trientine The aim of this study was to compare the pharmacokinetics of a new trientine tetrahydrochloride formulation (TETA 4HCl) with those of an established trientine dihydrochloride (TETA 2HCl) salt. Take this medication by mouth on an empty stomach, at least 1 hour before meals or 2 hours after meals as directed by your doctor, usually 2 to 4 times daily. Clipboard, Search History, and several other advanced features are temporarily unavailable. Cuvrior (trientine tetrahydrochloride) is a copper chelator indicated for the treatment of adult patients with stable Wilsons disease who are de-coppered and tolerant to penicillamine. It explains how the Agency assessed the medicine to recommend its authorisation in the EU and its conditions of use. WebTrientine tetrahydrochloride (TETA.4HCl) for the treatment of Wilsons disease ClinicalTrials.gov Identifier: NCT03539952 Participating countries: Belgium, Brazil, Denmark, France, Germany, Italy, Poland, Sweden, UK and USA Completion date: November 2021 https://www.clinicaltrials.gov/ct2/show/NCT03539952 Tell each of your healthcare providers about all your medical conditions, allergies, and all medicines you use. What Are Side Effects of Cuvrior? Zinc (usually zinc acetate dehydrate 75150mg/day in three divided doses) inhibits the enteric absorption of copper by stimulating intestinal and hepatic metallothioneins. The company provided data from the published literature, which show that trientine significantly increases copper elimination in the urine. WebThis medication is used to treat a certain inherited disorder ( Wilson's disease ). Trientine Tetrahydrochloride Pharmacokinetics The PubMed wordmark and PubMed logo are registered trademarks of the U.S. Department of Health and Human Services (HHS). Trientine and zinc have become major tools in the management of Wilsons disease. Cuvrior (trientine tetrahydrochloride) is a copper chelator indicated for the treatment of adult patients with stable Wilsons disease who are de-coppered and tolerant to penicillamine. Advise patients that copper level monitoring is required periodically during CUVRIOR therapy [see DOSAGE AND ADMINISTRATION and WARNINGS AND PRECAUTIONS]. Wilsons Disease is a rare inherited disorder of copper transport that leads to excess copper accumulation. We comply with the HONcode standard for trustworthy health information. It is unknown if this medication passes into breast milk. WebTablets: 300 mg of trientine tetrahydrochloride (equivalent to 150 mg of trientine), oblong, yellow coated, functionally scored, printed with OL75 on each side of score line in black ink. In those who had no previous treatment with H2Pen both agents induced a very large cupruresis, while in patients effectively decoppered by prolonged treatment, the cupruretic response was lower. This material is provided for educational purposes only and is not intended for medical advice, diagnosis or treatment. For centrally authorised veterinary medicines authorised or updated from February 2022, see theVeterinary Medicines Information website. It is not known whether trientine passes into breast milk or if it could harm a nursing baby. WebThe hydrochloride salt of TETA, referred to as trientine hydrochloride, is a chelating agent that is used to bind and remove copper in the body to treat Wilson's disease, particularly in those who are intolerant to penicillamine. Ramli FF, Hashim SAS, Raman B, Mahmod M, Kamisah Y. Some recommend trientine as first-line treatment, but experience with penicillamine is more extensive. Indications and dose Show For trientine Show For Cufence Show For Cuprior Interactions View interactions for trientine Side-effects For trientine Take this medication by mouth on an empty stomach, at least 1 hour before meals or 2 hours after meals as directed by your doctor, usually 2 to 4 times daily. Cuprior is used in patients who cannot take D-penicillamine, another medicine for this condition. Swallow the whole or split tablet without crushing or chewing. The active substance in Cuprior, trientine, is a chelating agent. Dosage for Cuvrior Common adverse reactions include abdominal pain, change of bowel habits, rash, alopecia, and mood swings. other drugs taken orally at the same time. WebA retrospective survey of the clinical experience of trientine in the management of Wilsons disease has been conducted with two different trientine formulations: a nationally produced tetrachloride (TETA 4HCl) formulation and trientine dihydrochloride (TETA 2HCL). Dosage form: oral capsule (250 mg) Trientine hydrochloride capsules, USP contain inactive ingredients stearic acid, the capsule shell contains D & C Red 28, FD & C Blue 1, gelatin, and titanium dioxide. Before having surgery, tell your doctor or dentist about all the products you use (including prescription drugs, nonprescription drugs, and herbal products). However, iron deficiency may develop, especially in children and menstruating or pregnant women, or as a result of the low copper diet recommended for Wilsons disease. Because many drugs are excreted in human milk, caution should be exercised when trientine hydrochloride is administered to a nursing mother. Before using this medication, tell your doctor or pharmacist your medical history. Trientine Similar to d-penicillamine, trientine should be administered either 1h before or 2h after meals, as food inhibits its absorption. In female rats, pulmonary lesions occurred at 175 mg/kg/day (lower than the maximum recommended dose based on body surface area). Trientine 2007;369(9559):397408. Experience on switching trientine formulations in Wilson disease: Efficacy and safety after initiation of TETA 4HCl as substitute for TETA 2HCl. Trientine tetrahydrochloride is more rapidly absorbed and provides greater systemic exposure of the active moiety trientine compared with trientine dihydrochloride. Bottles of 100 NDC 31722-683-01 FDA pregnancy category C. It is not known whether trientine will harm an unborn baby. Its principal role is as an alternative chelator for patients intolerant of d-penicillamine. Nat Rev Dis Primers. You may report side effects to Health Canada at 1-866-234-2345. If you are taking the capsule form of this medication, swallow the capsules whole with water. The aim of this study was to compare the pharmacokinetics of a new trientine tetrahydrochloride formulation (TETA 4HCl) with those of an established trientine dihydrochloride (TETA 2HCl) salt. TRIENTINE TETRAHYDROCHLORIDE Data sources include IBM Watson Micromedex (updated 3 July 2023), Cerner Multum (updated 10 July 2023), ASHP (updated 10 July 2023) and others. Wilson disease (WD) is a rare autosomal recessive genetic disorder that results in the pathological accumulation of copper in the liver, brain and other organs [ 1, 2, 3, 4 ]. The exact mechanism of action is not clear. WebTrientine tetrahydrochloride (brand name Cuprior) was approved for medical use in the European Union in September 2017. Package insert / product label This product is no longer an orphan medicine. WebHow to use Trientine HCL. This difference is not considered clinically meaningful for CUVRIOR. Business - enquiry@nuraychemicals.com Other Info - info@nuraychemicals.com. WebTrientine tetrahydrochloride | C6H22Cl4N4 | CID 71434 - structure, chemical names, physical and chemical properties, classification, patents, literature, biological activities, safety/hazards/toxicity information, supplier lists, and more. Role of Trientine in Hypertrophic Cardiomyopathy: A Review of Mechanistic Aspects. Trientine tetrahydrochloride Currently, in countries where available (limited), TN is usually used in patients with ADRs during DPA treatment, as the negative effects of DPA mostly resolve with TN and do not recur (EASL, 2012). In a retrospective study in five patients, trientine 5001000mg/day as a single dose was effective in controlling Wilsons disease [15]. Skip the missed dose if it is almost time for your next scheduled dose. As a copper chelator, trientine when taken with meals also reduces enteric copper absorption and promotes urinary copper excretion; the usual daily dose is 5001500mg/day, in two to four divided doses. However, on limited clinical experience, the recommended initial dose of trientine hydrochloride capsules is 500 to 750 mg/day for pediatric patients and 750 to 1250 mg/day for adults given in divided doses two, three or four times daily. Trientine tetrahydrochloride is more rapidly absorbed and provides greater systemic exposure of the active moiety trientine compared with trientine dihydrochloride. Differences between TETA 4HCl and TETA 2HCl in the levels of the two main mono- and diacetylated metabolites were less than seen for trientine. Introduction The capsules are imprinted with black ink containing black iron oxide, potassium hydroxide, propylene glycol, shellac, strong ammonia solution. It not only acts as a chelating agent and promotes urinary copper excretion, but also inhibits intestinal copper absorption through metallothionein induction. A chelating agent works by removing a heavy metal (such as lead, mercury, or copper) from the blood. To help you remember, take it at the same times each day. Select one or more newsletters to continue. Changes since initial authorisation of medicine, Initial marketing-authorisation documents, Committee for Medicinal Products for Human Use, Meeting highlights from the Committee for Medicinal Products for Human Use (CHMP) 18-21 April 2017, Send a question to the European Medicines Agency, International non-proprietary name (INN) or common name, Anatomical therapeutic chemical (ATC) code, Date of issue of marketing authorisation valid throughout the European Union. The effectiveness of CUVRIOR for the treatment of adult patients with stable Wilsons disease who are decoppered and tolerant to penicillamine was demonstrated in a phase 3 trial (Trial 1). Triethylenetetramine It was originally designated an orphan medicine on 19 March 2015. Trientine is used to treat this inherited condition in people who cannot take penicillamine. In the US - Call your doctor for medical advice about side effects. In general, the effects of trientine hydrochloride on urinary copper excretion are similar to those of equimolar doses of penicillamine, although in one study they were significantly smaller. Trientine Tetrahydrochloride ScienceDirect is a registered trademark of Elsevier B.V. ScienceDirect is a registered trademark of Elsevier B.V. Rosenberg's Molecular and Genetic Basis of Neurological and Psychiatric Disease (Fifth Edition), Meyler's Side Effects of Drugs (Sixteenth Edition), Immunosuppression, rheumatic diseases, multiple sclerosis, and Wilsons disease, Drugs During Pregnancy and Lactation (Third Edition), European Association for the Study of the Liver 2012, Oral Chelator Treatment of Wilson Disease, Clinical and Translational Perspectives on WILSON DISEASE, Roberts and Schilsky, 2008; EASL, 2012; PDR, 2016, Scheinberg et al., 1987; Dubois et al., 1990; Dahlman et al., 1995; Weiss et al., 2013a, Roberts and Schilsky, 2008; EASL, 2012; Weiss et al., 2013a, Trientine for Wilson Disease: Contemporary Issues, A worldwide yearly survey of new data in adverse drug reactions, International Encyclopedia of Public Health, Chelating Agents as Therapeutic CompoundsBasic Principles, Chelation Therapy in the Treatment of Metal Intoxication, , as an alternative for the patients presenting intolerance toward D-penicillamine. Trientine A chelating agent works by removing a heavy metal (such as lead, mercury, or copper) from the blood. HETERO LABS LIMITED The trientine hydrochloride group received d-penicillamine for an average of 4 years as compared to an average of 10 years for the nontreated group. If you want to ask a question or request information from EMA, please Send a question to the European Medicines Agency. Follow all directions on your medicine label and package. Since Cuprior is a hybrid medicine, its benefits and risks are taken as being the same as the reference medicine's. The lesions included alveolar fibrosis, bronchiolar hyperplasia/ epithelialization of alveolar wall, focal chronic interstitial pneumonitis, bronchiolar epithelium hypertrophy, alveolar hemorrhage (males only), necrosis/regenerative hyperplasia of terminal bronchioles (males only), and acute interstitial pneumonitis (males only). Patients (especially women) should be closely monitored for evidence of iron deficiency anemia. Retrospectively, he compared these patients to an additional group of 12 patients with Wilsons disease who were both tolerant of and controlled with d-penicillamine therapy, but who failed to continue any copper chelation therapy. For patients who have difficulty swallowing the tablet whole, the scored tablet can be divided into two equal halves. WebTrientine tetrahydrochloride (TETA.4HCl) for the treatment of Wilsons disease ClinicalTrials.gov Identifier: NCT03539952 Participating countries: Belgium, Brazil, Denmark, France, Germany, Italy, Poland, Sweden, UK and USA Completion date: November 2021 https://www.clinicaltrials.gov/ct2/show/NCT03539952 We comply with the HONcode standard for trustworthy health information. Your condition will not improve any faster, and your risk of side effects will increase. It is indicated for the treatment of Wilsons disease in adults, adolescents and children five years of age or older who are intolerant to D-penicillamine therapy. The maintenance dosage of TN should be 7501000mg/day (depending on copper metabolism). Methods: You may notice worsening in certain symptoms of Wilson's disease (such as problems with speech/swallowing/coordination, uncontrolled movements) when you start taking trientine as the medication begins to work to reduce the copper levels in your body. As a typical orphan drug, TN has been licensed in WD treatment without any studies on its absorption, excretion, or metabolism or formal tests on toxicity (Walshe, 1973); until now few data have existed about its pharmacokinetics (EASL, 2012). The dosage varied from 450 to 2400 mg per day. Trientine Tetrahydrochloride FDA Approves Cuvrior (trientine tetrahydrochloride) for the Treatment of Wilsons disease, Orphalan Announces Positive Top Line Data with Trientine Tetrahydrochloride for Maintenance Patients with Wilsons Disease. Wilson's disease is a genetic metabolic defect that causes excess copper to build up in the body. Data sources include IBM Watson Micromedex (updated 3 July 2023), Cerner Multum (updated 10 July 2023), ASHP (updated 10 July 2023) and others. However, the relevance of this finding to safety risk in patients with Wilsons disease is unknown. Do not drink milk, eat food, or take any other medications for at least 1 hour after you take trientine. It not only acts as a chelating agent and promotes urinary copper excretion, but also inhibits intestinal copper absorption through metallothionein induction. Cuvrior (trientine tetrahydrochloride) is a copper chelator indicated for the treatment of adult patients with stable Wilsons disease who are de-coppered and tolerant to penicillamine. Data on the pharmacokinetics of trientine hydrochloride is not available. Careers. Wilsons Disease is a rare inherited disorder of copper transport that leads to excess copper accumulation. Trientine is an effective treatment for Wilsons disease. What Are Side Effects of Cuvrior? The mechanism of pulmonary toxicity was not clearly established as due to copper depletion (primary pharmacology) or due to other drug activity. Cuprior is available as 150 mg tablets. Trientine may also be used for purposes not listed in this medication guide. Chelation treatment with trien can induce zinc depletion when the ligand is in excess with respect to the amount required to chelate the target copper metal ion. Trientine is an effective treatment for Wilsons disease. Other drugs may interact with trientine, including prescription and over-the-counter medicines, vitamins, and herbal products. Other reported clinical experience is insufficient to determine differences in responses between the elderly and younger patients. Store in the refrigerator, do not freeze.