Results 21 to 30 of about 1,630 (179)

Copper and zinc concentrations in the breast milk of mothers undergoing treatment for Wilson’s disease: a prospective study

BMJ Paediatrics Open, 2021
Objective To evaluate the concentrations of copper and zinc in the breast milk of mothers undergoing treatment for Wilson’s disease (WD) and clarify whether they can safely breast feed their infants.Design This was an observational and prospective study ...
Yasuhiro Sato, Hiroko Kodama, Yasumi Anan, Yoichi Izumi, Yasumitsu Ogra   +4 more
doaj   +2 more sources

Case Report: Beyond the initial diagnosis: a case of Wilson's disease presenting as acute decompensated cirrhosis [PDF]

Frontiers in Medicine
Wilson disease is a rare inherited disorder of copper metabolism that can cause progressive hepatic injury, cirrhosis, and, in severe cases, acute liver failure.
Aun Ali Bangash, Muhammad Ali Bangash, Chloe Cheree Harris, Veronica O’Brien, Learsi De Hoyos, Jose Campo Maldonado   +5 more
doaj   +2 more sources

Relative Exchangeable and Exchangeable Copper: Emerging New Biomarkers for Diagnosis and Therapy Monitoring in Wilson's Disease. [PDF]

Liver Int
ABSTRACT Background and Aims Wilson's disease (WD) is a genetic disorder of copper metabolism in which early diagnosis remains challenging, particularly in acute liver failure (ALF). Relative exchangeable copper (REC) and exchangeable copper (CuEXC) are emerging biomarkers for diagnosis and monitoring, but data from larger cohorts are limited.
Köhrer S, Rusev A, Zimmer H, Wolf S, Langel J, Langel A, Longerich T, Michl P, Niesert M, Fichtner A, Mohr I.   +10 more
europepmc   +2 more sources

Trientine Hydrochloride (MAR-Trientine)

Canadian Journal of Health Technologies, 2021
CADTH recommends that MAR-Trientine should be reimbursed by public drug plans for the treatment of patients with Wilson's disease if certain conditions are met. MAR-Trientine should only be covered to treat patients who have previously tried and demonstrated intolerance to d-penicillamine. MAR-Trientine should only be reimbursed if ...
null Reimbursement Team
openaire   +4 more sources

The Mechanism of Excretion of Trientine from the Rat Kidney: Trientine is not Recognized by the H+/Organic Cation Transporter

Journal of Pharmacy and Pharmacology, 1997
AbstractTrientine dihydrochloride is used to treat Wilson's disease by chelating copper and increasing its urinary excretion. The mechanism of renal excretion of trientine has been investigated in-vivo and in-vitro.Trientine clearance in the rat was significantly faster than creatinine clearance.
M, Kobayashi, R, Tanabe, M, Sugawara, K, Iseki, K, Miyazaki   +4 more
openaire   +3 more sources

Management of Wilson disease across Europe: an international physician-oriented survey by the ERN-RARE Liver group [PDF]

Orphanet Journal of Rare Diseases
Background Wilson disease (WD) is a rare disorder resulting in copper overload. Diagnosis and treatment are complex and highly specialized. We aimed to investigate the management of WD across Europe in line with the mission and framework of the European ...
Frederik Teicher Kirk, Karina Stubkjær Rewitz, Zoe Mariño, Eduardo Couchonnal, Nicolas Lanthier, Wiebke Papenthin, Marina Berenguer, Aurelia Poujois, Dominique Debray, Aftab Ala, Luis García-Villarreal, Tudor Lucian Pop, Gerald Denk, Piotr Socha, Thomas Damgaard Sandahl   +14 more
doaj   +2 more sources

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

, 2023
Aims: Hypertrophic cardiomyopathy (HCM) is characterised by left ventricular hypertrophy (LVH), myocardial fibrosis, enhanced oxidative stress and energy depletion.
Neubauer, Stefan, Matthias Schmitt, Baroja, Olatz, Spowart, Catherine, Masliza Mahmod, Olatz Baroja, Miller, Christopher, Beatriz Duran, Carly Vaughan, Dannii Clayton, Singh, Anvesha, Farrant, John; id_orcid, Vaughan, Carly, Josephine H Naish, Reid, Anna, Akhtar, Mohammed, Clayton, Dannii, Garratt, Clifford, Naish, Josephine, Raman, Betty, Harrington, Chris F., Anvesha Singh, Schmitt, Matthias, Sanjay K Prasad, Cooper, Robert M, Prasad, Sanjay K, Dodd, Susanna, Prasad, Sanjay K., Duran, Beatriz, Robert M Cooper, Chris Harrington, Emma Bedson, Mahmod, Masliza, TEMPEST, Investigators, Betty Raman, Bedson, Emma, Zakariye Ashkir, Clifford Garratt, Cooper, Robert, Farrant, John, Naish, Josephine H, Harrington, Chris, John Farrant, Valkovič, Ladislav, Ladislav Valkovič, Ashkir, Zakariye, Anna Reid, Christopher A Miller, Susanna Dodd, Mohammed Akhtar, Catherine Spowart, Stefan Neubauer, Miller, Christopher A   +52 more
core   +5 more sources

Trientine tetrahydrochloride (Cuprior)

, 2019
Use of trientine tetrahydrochloride for the treatment of Wilson's disease.
Rupert Purchase (16039694)
core   +2 more sources

Benefits and drawbacks of current copper chelators in Wilson disease [PDF]

Romanian Journal of Neurology, 2023
Background. Wilson disease (WD) is an autosomal-recessive disorder of copper metabolism, caused by mutations in the ATP7B gene, which codes for a membrane-bound copper-binding ATPase.
Ioan-Cristian Lupescu, Maria Speranta Iacob, Razvan Iacob, Daniela Anghel, Octavia Adriana Dulamea, Crisanda Vilciu, Liliana Gheorghe   +6 more
doaj   +1 more source

Analyzing the Therapeutic Efficacy of Bis-Choline-Tetrathiomolybdate in the Atp7b^−/− Copper Overload Mouse Model

Biomedicines, 2021
Bis-choline-tetrathiomolybdate, introduced as WTX101 (now known as ALXN1840), is a first-in-class copper-protein-binding agent for oral therapy of Wilson’s disease.
Philipp Kim, Chengcheng Christine Zhang, Sven Thoröe-Boveleth, Eva Miriam Buhl, Sabine Weiskirchen, Wolfgang Stremmel, Uta Merle, Ralf Weiskirchen   +7 more
doaj   +1 more source