Results 11 to 20 of about 2,712 (224)

Adherence, satisfaction, and quality of life in Wilson disease patients after switching to trientine tetrahydrochloride: observational data from a dual cohort study. [PDF]

Front Pharmacol
BackgroundWilson disease (WD) is a rare genetic disorder requiring lifelong treatment. Adherence to therapy is thus of utmost importance. We aimed to assess the change in treatment adherence and satisfaction in adult patients with WD after transitioning ...
Medici V, Cazzagon N, Battezzati PM, Crosignani A, Civolani A, Bonaiuto E, D'Incà R, Vargiu E, Tedone F, Lopatriello S, Zuin M.   +10 more
europepmc   +3 more sources

TDMQ20 as A Drug Candidate for Wilson's Disease: Comparison with D-Penicillamine, Trientine, and Tetrathiomolybdate In Vitro and In Mice. [PDF]

Pharmaceutics
Background/Objectives: The lifelong treatment of Wilson’s disease (WD) currently relies on copper chelators with relatively poor metal specificity, which frequently exhibit serious adverse effects.
Zhu Y, Peng W, Liu G, Li L, Zhou Z, Nguyen M, Robert A, Liu Y, Meunier B.   +8 more
europepmc   +3 more sources

Trientine Tetrahydrochloride, From Bench to Bedside: A Narrative Review [PDF]

Drugs
Trientine tetrahydrochloride (TETA-4HCl, Cuvrior®) is a copper chelating agent with the active moiety triethylenetetramine (trientine), developed by Orphalan, Inc. to address the unmet needs in the treatment of Wilson disease.
Kamlin COF, M Jenkins T, L Heise J, S Amin N.   +3 more
exaly   +3 more sources

Exploring the potential of trientine tetrahydrochloride in the treatment of Wilson disease

Health Sciences Review, 2023
Wilson disease is one of the uncommon, hereditary, slowly progressing, and autosomal recessive diseases that cause motion disorders. The pathogenesis of Wilson disease is attributed to the accumulation of copper in different parts of body mainly the ...
Nikita Sharma, Debashree Debasish Das, Pooja A Chawla   +2 more
doaj   +2 more sources

Comparative analysis of clinical outcomes and safety profile of trientine and d-penicillamine in the management of Wilson’s disease: A systematic review and meta-analysis

Rare
Introduction: Wilson disease (WD) is a rare metabolic disorder of copper metabolism, requiring life-long therapy, usually with D-penicillamine or trientine. This review compares their clinical effectiveness and safety.
Hafiz Muhammad Ehsan Arshad, Muhammad Zain Raza, Musab Maqsood, Muhammad Omais, Muhammad Hashim Faisal, Ali Ahmad Nadeem   +5 more
doaj   +2 more sources

Role of Trientine in Hypertrophic Cardiomyopathy: A Review of Mechanistic Aspects

Pharmaceuticals, 2022
Abnormality in myocardial copper homeostasis is believed to contribute to the development of cardiomyopathy. Trientine, a copper-chelating drug used in the management of patients with Wilson’s disease, demonstrates beneficial effects in patients with ...
Fitri Fareez Ramli, Syed Alhafiz Syed Hashim, Betty Raman, Masliza Mahmod, Yusof Kamisah   +4 more
doaj   +2 more sources

Developing an analytical method for quantification of trientine based on modified silver nanoparticles

BMC Chemistry, 2023
Trientine or (N,N´-bis(2-aminoethyl)-1,2-ethanediamine (TETA) is a copper chelator and used in Wilson’s disease, is aliphatic amine that does not have UV absorbing groups.
Mahsa Khodadadi, Ali Shayanfar
doaj   +2 more sources

Rationale and design of a randomised trial of trientine in patients with hypertrophic cardiomyopathy [PDF]

Heart, 2023
Aims Hypertrophic cardiomyopathy (HCM) is characterised by left ventricular hypertrophy (LVH), myocardial fibrosis, enhanced oxidative stress and energy depletion. Unbound/loosely bound tissue copper II ions are powerful catalysts of oxidative stress and
Akhtar, Mohammed, Ashkir, Zakariye, Baroja, Olatz, Bedson, Emma, Clayton, Dannii, Cooper, Robert M, Dodd, Susanna, Duran, Beatriz, Farrant, John, Garratt, Clifford, Harrington, Chris, Mahmod, Masliza, Miller, Christopher A, Naish, Josephine H, Neubauer, Stefan, Prasad, Sanjay K, Raman, Betty, Reid, Anna, Schmitt, Matthias, Singh, Anvesha, Spowart, Catherine, TEMPEST, Investigators, Valkovic, Ladislav, Vaughan, Carly   +23 more
core   +3 more sources

Advances in Treatment of Wilson Disease [PDF]

Tremor and Other Hyperkinetic Movements, 2018
Background: Wilson disease (WD) is an inherited neurometabolic disorder that results in excessive copper deposition in the liver and the brain, affecting children and young adults. Without treatment the disease is invariably fatal.
Annu Aggarwal, Mohit Bhatt
doaj   +3 more sources

Case Report: Childhood Erythrocytosis due to Hypermanganesemia Caused by Homozygous <i>SLC30A10</i> Mutation. [PDF]

Front Hematol
We present a rare case of erythrocytosis due to a homozygous SLC30A10 mutation, causative of Hypermanganesemia with Dystonia, Polycythemia, and Cirrhosis (HMDPC).
Coppola T, Hughes H, Finch P, Hess J, Wu S, Prada C, Miethke AG, Zhang W, Kalfa TA.   +8 more
europepmc   +2 more sources