trientine / Generic mfg. - LARVOL DELTA

Therapeutic Management of Wilson Disease in Clinical Practice: Multicenter Retrospective Real-World Data from Germany. (PubMed, Z Gastroenterol) - "Chelating agents available include D-penicillamine (DPA) and trientine (TETA-2HCl and TETA-4HCl). These real-world data describe WD treatment management at specialized German centers. The observed dose changes during formulation switches are descriptive and hypothesis-generating, but prospective studies with clinical outcome data are needed before any switch factor can be recommended for routine practice."

Journal • Real-world evidence • Retrospective data • Genetic Disorders • Metabolic Disorders • Movement Disorders • Rare Diseases

Comparative Pharmacokinetics of Trientine Dihydrochloride Capsules: A Randomized, Open-Label, Reference-Controlled, Two-Period Crossover Study in Healthy Volunteers. (PubMed, Drugs R D) - "At the administered strengths, AUC0-t was similar between the formulations, whereas the upper confidence limit for Cmax slightly exceeded 125.00%. Faster absorption and higher dose-normalized exposure with the test formulation indicate formulation-dependent pharmacokinetic differences and support cautious use of bioequivalence terminology outside the specific regulatory assessment context."

Journal • PK/PD data • Genetic Disorders • Metabolic Disorders • Movement Disorders

Wilson Disease Beyond Youth: A Case of Late-Onset Diagnosis in a 54-Year-Old Man With Advanced Fibrosis and Markedly Elevated Hepatic Copper (ACG 2026) - "Given concern for Wilson disease, he was started on copper directed therapy with trientine and dietary copper restriction...Wilson disease should remain on the differential for unexplained transaminitis in adults of any age, especially with a suggestive family history. Early recognition allows copper directed therapy and may prevent progression."

Clinical • Metastases • Diabetes • Fibrosis • Genetic Disorders • Hepatology • Immunology • Inflammation • Metabolic Disorders • Metabolic Dysfunction-Associated Steatotic Liver Disease • Movement Disorders • CP

Virtual Simulation CME Improves Diagnosis and Management of Wilson Disease (ACG 2026) - "In maintenance management (Case 2), adoption of trientine tetrahydrochloride increased by 19% (P < .001)... A total of 9,061 learners participated in the program. Performance data for the target audience demonstrated significant gains across core learning objectives (LO): Diagnostic Workup (LO1): Gastroenterologists/Hepatologists showed a 16% improvement in the diagnostic workup for suspected liver disease (P < .001). Significant lifts were noted in recognizing extrahepatic cues, specifically requesting slit-lamp exams (+18%) and liver ultrasound (+16%)."

Genetic Disorders • Hepatology • Metabolic Disorders • Movement Disorders

The utility of whole exome sequencing in diagnosing Wilson disease: A case report. (PubMed, JPGN Rep) - "The patient was started on trientine, and ALT normalized within 3 months, with increased urinary copper excretion. This case underscores how WD can mimic autoimmune hepatitis and metabolic dysfunction-associated steatotic liver disease (MASLD), and how diagnosis may rely on integrating histologic findings, genetic data, and therapeutic response."

Journal • Autoimmune Hepatitis • Genetic Disorders • Hepatology • Immunology • Inflammation • Metabolic Disorders • Metabolic Dysfunction-Associated Steatotic Liver Disease • Movement Disorders • ATP7B

Trientine for hypertrophic cardiomyopathy: a phase 2 trial. (PubMed, Eur Heart J) - P2 | "Among patients with hypertrophic cardiomyopathy, treatment with trientine resulted in a significantly greater reduction in left ventricular mass indexed to body surface area than placebo. (Funded by NIHR; TEMPEST ClinicalTrials.gov number, NCT04706429)."

Journal • P2 data • Cardiomyopathy • Cardiovascular • Fibrosis • Hypertrophic Cardiomyopathy • Immunology

SUBCLİNİCAL EMOTİONAL REGULATİON DEFİCİTS İN CHİLDREN WİTH WİLSON DİSEASE: FOCUS ON IMPULSE CONTROL (ESPGHAN 2026) - "Chelation therapy consisted of D-penicillamine in 64% and trientine in 36% of patients. Integrating structured psychiatric evaluation and longitudinal neurocognitive monitoring into routine follow-up may enable early recognition of emotional dysregulation, inform individualized care and improve overall functioning and quality of life. Contact e-mail address [email protected]"

Genetic Disorders • Hepatology • Metabolic Disorders • Movement Disorders

Pediatric Wilson Disease in Sudan: A Rare Case, Sudan Conflict and Diagnostic Challenges. (PubMed, Clin Case Rep) - "Initial therapy with D-penicillamine and then zinc was administered, but it failed to prevent neurological decline...Moreover, the only alternate therapy, trientine, in all its forms, was not available at the time. Wilson disease, thought rare, occurs in African children but remains underdiagnosed due to resource constraints. This case highlights diagnostic challenges, the critical role of clinical suspicion, and urgent need for accessible diagnostic and affordable therapies in low-resource settings to improve outcome."

Journal • Genetic Disorders • Hematological Disorders • Metabolic Disorders • Movement Disorders • Pediatrics • Psychiatry • Rare Diseases • Thrombocytopenia • ATP7B

A novel trientine tetrahydrochloride formulation administered once-a-day for people living with Wilson disease: results from a phase 2 study (EASL 2026) - P2 | "People living with WD report positive impact on convenience and adherence after 28d of QD TETA-4HCl whilst demonstrating favourable tolerability and biomarker stability."

Late-breaking abstract • P2 data • Genetic Disorders • Hepatology • Metabolic Disorders • Movement Disorders • Rare Diseases

The pharmacokinetic profile of a new trientine tetrahydrochloride formulation supports simpler once-daily dosing for the treatment of wilson disease: results of the triumph-3 study (EASL 2026) - P1 | "The new 300mg trientine base formulation of TETA 4HCl administered QD with a TDD of 900mg was well tolerated and demonstrated comparative PK profile to 900mg of approved TETA 4HCl administered BID."

PK/PD data • Genetic Disorders • Hepatology • Infectious Disease • Metabolic Disorders • Movement Disorders

Non-ceruloplasmin bound copper measured by speciation assay: response to therapy over 12 months in real world setting of newly diagnosed Wilson disease patients (EASL 2026) - P, P3 | "Most common first line therapy was zinc salts (N=6) followed by d-penicillamine (N=2) and trientine (N=1). NCC-Sp is elevated in newly diagnosed WD and falls in response to treatment in the first 6 months. Liver enzymes are modestly elevated at diagnosis and remain static over 12 months. NCC-Sp has the potential, if confirmed in future studies, to be an accurate and precise biomarker to monitor de-coppering and facilitate response-based titration of medication."

Clinical • Real-world • Real-world evidence • Genetic Disorders • Hepatology • Metabolic Disorders • Movement Disorders

Non-ceruloplasmin bound copper measured by speciation assay: prospective evaluation over 12 months in real world setting of unstable Wilson disease patients (EASL 2026) - P, P3 | "Baseline treatments included trientine (N=29), zinc salts (N=8) and d-penicillamine (N=6). Observed NCC-Sp reductions support the potential clinical utility of NCC-Sp as a treatment responsive, clinically meaningful biomarker for monitoring response to, adherence and guiding treatment decisions in biochemically unstable WD."

Clinical • Real-world • Real-world evidence • Genetic Disorders • Hepatology • Metabolic Disorders • Movement Disorders

Understanding Wilson disease beyond traditional phenotypes: cross-sectional analysis of study entry data from an international registry (iWilson) (EASL 2026) - P | "Distribution of WD therapy from 369 records: d-penicillamine (DPA; 41.6%), zinc salts (28.8%), trientine tetrahydrochloride (16.2%), trientine dihydrochloride (8.5%), unspecified trientine (8.1%). Baseline data from iWD gathered across geographic boundaries, captures real-time commonalities and differences of cross-sectional disease status and treatment experience from more than 380 people living with WD. Future longitudinal analyses may enable comparison of regional diagnostic and therapeutic approaches and outcome assessment across healthcare systems over time, supporting the evolution toward biology-informed care."

Fibrosis • Genetic Disorders • Hepatology • Immunology • Metabolic Disorders • Movement Disorders • Rare Diseases

Variation of management in Wilson's disease in England: results from the first national audit (EASL 2026) - "D-penicillamine was the most commonly prescribed medication with 57% also prescribed pyridoxine (75% daily dosage compared to weekly). 37% of patients were on some form of Trientine with the remaining on zinc... Despite the high usage of published guidance there remained significant disparity in management regimes, in particular with treatment monitoring and titration. We will engage with clinicians at participating centres to explore the rationale leading to current practice. We hope to then achieve a level of standardisation of management of WD in line with current recommendations to elevate patient care."

Fibrosis • Genetic Disorders • Hepatology • Immunology • Metabolic Disorders • Movement Disorders

A Case of Wilson’s Disease With Initial Unilateral Chorea and Contralateral MRI Changes (AAN 2026) - "The patient was restarted on her maintenance trientine regimen of 250 mg three times daily and zinc, with marked improvement of chorea. Trials of clonazepam, deutetrabenazine, and valbenazine produced no significant benefit...Conclusions This case represents one of the first reported instances of initial unilateral neurological manifestations of Wilson’s disease accompanied by contralateral MRI changes. It underscores the importance of recognizing atypical neurological presentations of Wilson’s disease."

Clinical • Diabetes • Genetic Disorders • Hepatology • Immunology • Metabolic Disorders • Movement Disorders

Transition from zinc salts to trientine tetrahydrochloride in a cohort of adult patients with Wilson disease: the ZICUP study. (PubMed, Orphanet J Rare Dis) - No abstract available

Journal • Genetic Disorders • Metabolic Disorders • Movement Disorders

Maternal and Fetal Outcomes of Pregnancies in Wilson's Disease: A Single-Centre Real-Life Experience. (PubMed, Turk J Gastroenterol) - "With careful monitoring and management by medical professionals, pregnant women diagnosed with WD can still achieve successful pregnancies and improve their quality of life.   Cite this article as: Demir K, Örmeci AÇ, Çavuş B, et al. Maternal and fetal outcomes of pregnancies in Wilson's disease: A singlecentre real-life experience. Turk J Gastroenterol. 2026;37(3):301-309."

Journal • Retrospective data • Genetic Disorders • Metabolic Disorders • Movement Disorders

KEEPING OUT THE COPPER: A CASE OF CARDIOMYOPATHY ASSOCIATED WITH WILSON'S DISEASE (ACC 2026) - "He was started on chelation therapy with trientine and zinc on initial diagnosis of Wilson's disease and achieved satisfactory cupriuresis for several years, until he was found to have inadequate urinary copper levels on a recent evaluation...He was also started on metoprolol and valsartan, with improvement of dyspnea on exertion and palpitations. Cardiomyopathy in Wilson's disease may be reversible following adequate copper chelation therapy and GDMT, but conduction disease may persist despite treatment."

Clinical • Cardiomyopathy • Cardiovascular • Genetic Disorders • Hepatology • Metabolic Disorders • Movement Disorders • Pulmonary Disease

Evaluation of novel assays of non-ceruloplasmin copper to monitor chelation treatment in patients with Wilson disease: Monitoring chelation treatment in Wilson disease. (PubMed, JHEP Rep) - P3 | "Non-ceruloplasmin by protein speciation and exchangeable copper have potential to guide chelation in WD patients on maintenance therapy. Specific target ranges should be established, and we hypothesize they may include values below normal ranges. Further studies are required to improve our understanding of the responses to dose changes and non-adherence and if standardization of sampling conditions can reduce visit-to-visit variability."

Journal • Genetic Disorders • Metabolic Disorders • Movement Disorders

TRADITiONAL: Trientine Tetrahydrochloride Administered Once a Day for the First Line Treatment of Wilson's Disease Patients. (clinicaltrials.gov) - P3 | N=38 | Not yet recruiting | Sponsor: Orphalan

New P3 trial • Genetic Disorders • Metabolic Disorders • Movement Disorders

Post-marketing safety signals of Wilson's disease therapies: evidence from FAERS and VigiBase. (PubMed, Orphanet J Rare Dis) - No abstract available

Journal • P4 data • Genetic Disorders • Hepatology • Metabolic Disorders • Movement Disorders

Trientine ameliorates bleomycin-induced pulmonary fibrosis in rats through copper chelation and modulation of the CTR1/LOX/COL pathway. (PubMed, Inflammopharmacology) - "Overall, TRI demonstrated potent antifibrotic, antioxidant, and anti-inflammatory properties, effectively protecting lung tissue against BLM-induced injury. These findings highlight copper chelation as a promising therapeutic strategy for pulmonary fibrosis and warrant further investigation into TRI's clinical potential."

Journal • Preclinical • Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases • IL6 • LOX • TGFB1 • TNFA

USE OF TRIENTINE IN PATIENTS FROM THE WILSON AEEH REGISTRY: INDICATIONS, RESPONSE, SIDE EFFECTS AND ADHERENCE TO TREATMENT (AEEH 2026) - No abstract available

Adherence • Adverse events • Clinical

Patient Burden in the Treatment of Wilson Disease in the United States: An Analysis of Real-World Health Insurance Claims Data from the Komodo database. (PubMed, Adv Ther) - "Estimated annual HCRU and treatment costs for patients with WD were lower than previously reported. These findings provide updated real-world insights into the economic burden of WD and highlight the cost implications of medication adherence in managing this rare disorder in the USA."

Journal • Real-world evidence • Reimbursement • US reimbursement • Genetic Disorders • Hepatology • Metabolic Disorders • Movement Disorders • Rare Diseases • Transplantation

Patient Preference Study: Standard of Care Versus Once-daily Trientine Tetrahydrochloride (clinicaltrials.gov) - P2 | N=10 | Completed | Sponsor: Orphalan | Active, not recruiting ➔ Completed

Trial completion • Genetic Disorders • Metabolic Disorders • Movement Disorders