trientine / Generic mfg. - LARVOL DELTA

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

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) - No abstract available

PK/PD data • Genetic Disorders • Hepatology • 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

A Case of Wilson's Disease With Initial Unilateral Chorea and Contralateral MRI Changes (AAN 2026) - "She had been on trientine and zinc for several years, but treatment had lapsed before presentation...Trials of clonazepam, deutetrabenazine, and valbenazine produced no significant benefit... 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

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

Further elucidation on the effect of food on the pharmacokinetics of trientine. (PubMed, Eur J Clin Pharmacol) - No abstract available

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

Wilson Disease Hiding in Plain Sight: A Case Report of Psychosis and Catatonia Revealing Underlying Liver Dysfunction. (PubMed, Reports (MDPI)) - "The patient was started on trientine and zinc sulfate, but progressive hepatic dysfunction necessitated liver transplantation...Timely, multidisciplinary evaluation is essential to avoid misdiagnosis and initiate appropriate therapy. Early intervention can significantly improve both psychiatric and medical outcomes in Wilson disease."

Journal • CNS Disorders • Genetic Disorders • Hepatology • Liver Failure • Mental Retardation • Metabolic Disorders • Movement Disorders • Psychiatry • Transplantation

New insights into the effects of dissolution profiles on the pharmacokinetics of trientine dihydrochloride. (PubMed, Eur J Clin Pharmacol) - "The pharmacokinetic profiles of TETA after administration of TETA-2HCl capsules with fast and slow dissolution characteristics were similar. Though the lower 90%-CI for AUC0-inf was outside the formal bioequivalence ranges, differences were small (9%) and not considered clinically relevant. A difference in dissolution profile did not affect copper parameters and tolerability."

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

Recent advances in the study of cuproptosis in gliomas. (PubMed, Int Immunopharmacol) - "Copper chelators, such as trientine and disulfiram, can regulate intracellular copper levels and induce glioma cell death, thereby enhancing the efficacy of chemotherapy. However, the specific mechanisms of cuproptosis and its clinical application in glioma treatment require further exploration. This field of study promises to offer novel insights and directions for developing more effective glioma therapies."

Journal • Review • Brain Cancer • Glioma • Oncology • Solid Tumor • FDX1 • LIAS

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

Enrollment closed • Genetic Disorders • Metabolic Disorders • Movement Disorders

Management of Wilson disease across Europe: an international physician-oriented survey by the ERN-RARE Liver group. (PubMed, Orphanet J Rare Dis) - "Overall, we found uniformity in the management of WD across European WD centers. Nevertheless, variations in key areas were identified, reflecting a lack of robust evidence, thus providing a guide for future research."

Journal • Genetic Disorders • Hepatology • Metabolic Disorders • Movement Disorders • Rare Diseases

Safety of penicillamine and trientine in the treatment of Wilson's disease: An analysis of the FDA Adverse Event Reporting System (FAERS) database. (PubMed, PLoS One) - "This study reveals the characteristics of AEs and potential associated risks in the clinical application of penicillamine and trientine, emphasizing individualized medication and vigilant monitoring strategies to provide guidance for safe medication use."

Adverse events • Journal • Alzheimer's Disease • CNS Disorders • Gastroenterology • Gastrointestinal Disorder • Genetic Disorders • Immunology • Metabolic Disorders • Movement Disorders

Rare liver diseases - Etiology, diagnosis and management: A review. (PubMed, Biomol Biomed) - "Lifelong copper chelation is recommended for Wilson disease, with trientine preferred for neurologic phenotypes. Supportive care in alpha-1 antitrypsin deficiency (A1ATD) is complemented by the investigation of molecular chaperones. Additionally, gene-directed therapies, gene editing, RNA-based approaches, and cell therapies show early promise but raise concerns regarding durability, safety, and ethical considerations, particularly for pediatric patients. In conclusion, implementing precision medicine frameworks that rely on standardized diagnostics, multicenter registries, and equitable access is crucial for facilitating earlier detection and translating mechanism-targeted therapies into sustainable, globally accessible benefits."

Journal • Review • Alpha-1 Antitrypsin Deficiency • Cholestasis • Genetic Disorders • Hepatology • Immunology • Metabolic Disorders • Movement Disorders • Pediatrics • Pulmonary Disease • Respiratory Diseases

Adherence, satisfaction, and quality of life in Wilson disease patients after switching to trientine tetrahydrochloride: observational data from a dual cohort study. (PubMed, Front Pharmacol) - "All quality-of-life (QoL) domains improved between T0 and T1. Adults with WD on maintenance therapy following a switch in therapy to TETA 4HCl some improvements in adherence, satisfaction and QoL were observed."

Clinical • HEOR • Journal • Observational data • Genetic Disorders • Metabolic Disorders • Movement Disorders

Late Onset Wilson Disease Presenting With Elevated Transaminases and Abnormal Ultrasound (ACG 2025) - "Repeat labs 6 weeks after initiating trientine show improvement of both AST and ALT, 45 U/L and 94 U/L respectively...In our patient, obesity initially directed the evaluation toward fatty liver disease...H&E StainFigure: Figure 2. Trichrome Stain"

Fibrosis • Genetic Disorders • Hepatology • Immunology • Inflammation • Liver Failure • Metabolic Disorders • Movement Disorders • Obesity • Ophthalmology • Psychiatry • ATP7B

Reprogramming the Tumor Microenvironment with Copper Chelators to Enhance Immunotherapy in Mesothelioma (IMIG 2025) - "Given their established safety profile and clinical use in childhood disorders, Cu chelators such as Trientine (TETA) represent a promising therapy to combine with immune checkpoint blockade (ICB) for mesothelioma. Ongoing phase I/II trials are evaluating Cu chelators in combination with anti-PD-1 (Pembrolizumab) and chemotherapy in breast cancer, or in combination with anti-GD2 (Dinutuximab)in neuroblastoma. These findings provide a strong rationale to assess the safety and feasibility of combining TETA with dual ICB (Ipilimumab/Nivolumab) in patients with mesothelioma."

Biomarker • IO biomarker • Tumor microenvironment • Breast Cancer • Gastric Cancer • Lung Cancer • Mesothelioma • Neuroblastoma • Oncology • Solid Tumor • CD8 • PD-L1

Wilson's Disease Initially Misdiagnosed as Metabolic Dysfunction-Associated Steatohepatitis in a Young Male (ACG 2025) - "He was started on Trientine HCl (500 mg AM & 750 mg PM). Our case underscores the complexity of diagnosis of Wilson's disease when hepatic steatosis is the only prominent finding...A broader differential, including Wilson's disease, should be considered in younger adults with elevated liver enzymes, even when imaging suggests MASH. Early diagnosis through comprehensive Cu studies allows for timely chelation therapy, helping to prevent irreversible liver or neurologic damage.Figure: Table 1: Laboratory ReportFigure: Table 2: Differential Diagnosis of Steatosis"

Fibrosis • Genetic Disorders • Hepatology • Immunology • Metabolic Disorders • Metabolic Dysfunction-Associated Steatohepatitis • Movement Disorders • ATP7B • CP

Behavioral and Quality of Life Challenges in Pediatric Wilson Disease: Insights From a Multisite International Registry (AACAP 2025) - "Participants on chelating agents (D-penicillamine or trientine) reported lower physical functioning (77.7 + 20.93, p = .004) and physical health (77.59 + 21.28, p = .005) than those on zinc (94.06 ± 7.35), possibly due to selection bias of using zinc for less ill patients. Children with WD experience internalizing symptoms (anxiety, depression, and somatic complaints) that impact their emotional well-being, social interactions, and academic performance. Children with WD experience internalizing symptoms (anxiety, depression, and somatic complaints) that impact their emotional well-being, social interactions, and academic performance. Females exhibiting more emotional distress may benefit from gender-sensitive interventions. The relationship between internalizing symptoms and academic struggles underscores the need to address mental health challenges early."

Clinical • HEOR • CNS Disorders • Depression • Genetic Disorders • Hepatology • Metabolic Disorders • Mood Disorders • Movement Disorders • Pediatrics • Psychiatry

Recommendations from the European Association for the Study of the Liver and the European Reference Network for Rare Liver Diseases Clinical Practice Guidelines for hepatolenticular degeneration (PubMed, Zhonghua Gan Zang Bing Za Zhi) - "Pharmacotherapy mainly includes chelating agents (such as penicillamine and trientine) and zinc salts...The diagnosis and treatment of acute liver failure with WD is extremely challenging, as the diagnosis is difficult and medical treatment cannot save life. The role of liver transplantation has been clearly recognized in the treatment of acute liver failure with WD, and it may also be considered in cases with neurological involvement."

Clinical guideline • Journal • Genetic Disorders • Hepatology • Liver Failure • Metabolic Disorders • Movement Disorders • Transplantation