Relapse following withdrawal of D-penicillamine from combination (D-penicillamine + zinc) therapy in hepatic Wilson disease. Long-term D-penicillamine (D-pen) therapy in Wilson disease (WD) has numerous adverse effects which advocates its withdrawal, but with an inherent risk of relapse. This prospective observational study was conducted with the objective of evaluating incidence of relapse
Effects of trientine and penicillamine on intestinal copper uptake: A mechanistic 64Cu PET/CT study in healthy humans. Trientine (TRI) and D-penicillamine (PEN) are used to treat copper overload in Wilson disease. Their main mode of action is thought to be through the facilitation of urinary copper excretion. In a recent study, TRI was noninferior to PEN despite lower 24-hour urinary copper
Trientine tetrahydrochloride versus penicillamine for maintenance therapy in Wilson disease (CHELATE): a randomised, open-label, non-inferiority, phase 3 trial. Wilson disease is an inherited disorder of copper transport. Whereas penicillamine is used therapeutically to re-establish copper balance, trientine is indicated for patients with penicillamine intolerance. We aimed to compare penicillamine with trientine tetrahydrochloride (TETA4) for maintenance therapy in patients with Wilson disease. We conducted a randomised, open-label, non-inferiority, phase 3 trial at 15 health-care centres across nine countries (patients were recruited from 13 of these health-care centres across Brazil, Europe, and the USA). We enrolled patients aged 18-75 years with stable Wilson disease who were treated
Penicillamine-induced degenerative dermopathy in a patient with Wilson's disease. Penicillamine is a chelator that has been used in Wilson's disease, cystinuria, rheumatoid arthritis and heavy metal intoxication. We report a case of a 31-year-old man presented with skin atrophy, purpura and milia on the hips and shoulders after taking penicillamine for 1.5 years. According to literature review , this type of penicillamine-associated cutaneous adverse effect belongs to degenerative dermopathy, which mostly occurs on bony prominences and points of pressure in patients with Wilson's disease or cystinuria. Withdrawal or reduction of drug dose can improve the features of degenerative dermopathy.
Penicillamine - Evaluation of the effects on reproduction, recommendation for classification Penicillamine - Evaluation of the effects on reproduction, recommendation for classification ..
Penicillamine An official website of the United States government Here's how you know Log inAccess keysNCBI HomepageMyNCBI HomepageMain ContentMain NavigationBookshelfSearch databaseBooksAll DatabasesAssemblyBiocollectionsBioProjectBioSampleBooksClinVarConserved DomainsdbGaPdbVarGeneGenomeGEO DataSetsGEO ProfilesGTRHomoloGeneIdentical Protein GroupsMedGenMeSHNLM and EffectsSummary of Use during LactationLimited information indicates that penicillamine is not detectable in breastmilk and no adverse effects have been reported among breastfed infants whose mothers were taking the drug. Copper and zinc levels in breastmilk are reduced in some mothers receiving penicillamine. Penicillamine has been used with apparent safety during nursing of several infants. Based on available
Combined sodium Dimercaptopropanesulfonate and zinc versus D-penicillamine as first-line therapy for neurological Wilson's disease. Even though recent research has achieved significant advancement in the development of therapeutic approaches for Wilson's diseases (WD), the current treatment options available for WD are still limited, especially for WD patients with neurological symptoms . This study is intended to compare the therapeutic approaches for WD patients with neurological symptoms receiving either combined sodium 2, 3-dimercapto-1-propane sulfonate (DMPS) and zinc treatment or D-penicillamine (DPA) monotherapy as first-line therapy, and identify the more effective therapeutic approach. The case records of 158 patients diagnosed with neurological WD were retrospectively analyzed
Urinary Abnormalities in Children and Adolescents with Wilson Disease Before and During Treatment with D-Penicillamine. Renal abnormalities can occur at any time point during the course of Wilson disease (WD). We aimed to fill a literature gap in this respect by studying urinary abnormalities in children and adolescents with WD. This study included 60 children with WD presenting to the Pediatric of the disease and during treatment with d-penicillamine. They have to be searched for, as early intervention may prevent progression to renal insufficiency.
Synthesis and Characterization of Controlled Nitric Oxide Release from S-Nitroso-N-Acetyl-d-Penicillamine Covalently Linked to Polyvinyl Chloride (SNAP-PVC) Polyvinyl chloride (PVC) is one of the most widely used polymers in medicine but has very poor biocompatibility when in contact with tissue or blood. To increase biocompatibility, controlled release of nitric oxide (NO) can be utilized to mitigate and reduce the inflammatory response. A synthetic route is described where PVC is aminated to a specified degree and then further modified by covalently linking -nitroso--acetyl-d-penicillamine (SNAP) groups to the free primary amine sites to create a nitric oxide releasing polymer (SNAP-PVC). Controllable release of NO from SNAP-PVC is described using photoinitiation from light emitting diodes
GanDouLing combined with Penicillamine improves cerebrovascular injury via PERK/eIF2α/CHOP endoplasmic reticulum stress pathway in the mouse model of Wilson’s disease We aim to investigate the function and mechanism of GanDouLing combinated with Penicillamine on cerebrovascular injury in Wilson's disease (WD). ELISA was performed to analyze the expression of vascular injury factors and markedly decreased in GanDouLing-Penicillamine group. The expression of caspase-3, caspase-12, PERK, eIF2α, and CHOP were obviously expressed in Wilson group, GanDouLing-Penicillamine suppressed apoptosis and endoplasmic reticulum (ER) stress. Our findings suggested that GanDouLing-Penicillamine improved cerebrovascular injury through PERK/eIF2α/CHOP ER stress pathway in the mouse model of WD.
Clinical efficacy and safety of Gandouling plus low-dose D-penicillamine for treatment of Wilson's disease with neurological symptoms. To investigate the effect and safety of Gandouling plus low-dose D-penicillamine for treating patients with Wilson's disease (WD) who have neurological symptoms. WD patients with neurological symptoms were divided into two groups: a treatment group (n = 53 ) and a control group (n = 50). The treatment group received anti-copper therapy with a combination of Gandouling and low-dose D-penicillamine (10 mg/kg), whereas the control group was with conventional dose D-penicillamine (20 mg/kg) monotherapy. The clinical efficacies, adverse reactions, and results of the various hematological and biochemical investigations were recorded and analyzed statistically. Overall
Analysis of penicillamine using Cu-modified graphene quantum dots synthesized from uric acid as single precursor A simple methodology was developed to quantify penicillamine (PA) in pharmaceutical samples, using the selective interaction of the drug with Cu-modified graphene quantum dots (Cu-GQDs). The proposed strategy combines the advantages of carbon dots (over other nanoparticles
Intracerebroventricular administration of the (1→6)-β-d-glucan (lasiodiplodan) in male rats prevents d-penicillamine-induced behavioral alterations and lipoperoxidation in the cortex Lasiodiplodan, an exocellular (1→6)-β-d-glucan of molecular weight >1.4 × 10 Da produced by MMPI strain of Lasiodiplodia theobromae (Pat.) Griffon & Maubl. (Brotyosphaeriaceae) is known to exhibit anti -proliferative activity on breast cancer cells (MCF-7), anticoagulant activity when sulfonylated, and reduction in transaminase activity when administered in rats. The effect of intracerebroventricular (I.C.V) injection of lasiodiplodan on neurotoxicity and behavioural changes induced by d-penicillamine was investigated. Twenty-four male Wistar rats were initially separated in groups of six and treated
Pre-Clinical Studies with D-Penicillamine as a Novel Pharmacological Strategy to Treat Alcoholism: Updated Evidences Ethanol, as other drugs of abuse, is able to activate the ventral tegmental area dopamine (VTA-DA) neurons leading to positively motivational alcohol-seeking behavior and use, and, ultimately to ethanol addiction. In the last decades, the involvement of brain-derived acetaldehyde in the prevention of ethanol relapse-like drinking behavior as well as in chronic alcohol consumption. In this sense, one of the most explored interventions has been the administration of D-Penicillamine (DP). These pre-clinical studies, that we critically summarize in this article, are considered a critical step for the potential development of a novel pharmacotherapeutic strategy for alcohol addiction treatment
D-penicillamine combined with inhibitors of hydroperoxide metabolism enhances lung and breast cancer cell responses to radiation and carboplatin via H2O2-mediated oxidative stress D-penicillamine (DPEN), a copper chelator, has been used in the treatment of Wilson's disease, cystinuria, and rheumatoid arthritis. Recent evidence suggests that DPEN in combination with biologically relevant copper
Combination Therapy with Low Copper Diet, Penicillamine and Gamma Knife Radiosurgery Reduces VEGF and IL-8 In Patients with Recurrent Glioblastoma Purpose: Vascular Endothelial Growth Factor (VEGF) and interleukin-8 (IL-8) appear important in tumor growth. Inthis study, we have investigated the effect of copper reduction along with gamma knife radiosurgery on IL-8 and VEGFin patients with recurrent glioblastoma multiforme (GBM). Materials and Methods: In a 3-month randomized clinicaltrial, patients with recurrent GBM were allocated randomly between intervention and placebo groups. Radiosurgerywas performed for both groups (Reference dose: 16-18 Gray, in one fraction). The intervention group received lowcopper diet and penicillamine while the patients in the placebo group continued
Chemosensitivity of U251 Cells to the Co-treatment of D-Penicillamine and Copper: Possible Implications on Wilson Disease Patients D-Penicillamine (PA), a copper chelator, and one of the recommended drugs for treatment of Wilson disease (WD) has been reported to worsen the symptoms of patients with neurologic presentations. However, the cause of this paradoxical response has not been fully
Comparative Analysis of Clinical Outcomes And Safety Profile of Penicillamine And Trientine in Management of Wilson's disease: A Systematic Review And Meta-Analysis. PROSPEROInternational prospective register of systematic reviews Print | PDFComparative Analysis of Clinical Outcomes And Safety Profile of Penicillamine And Trientine in Management of Wilson’s disease: A Systematic Review And Meta . Therefore, automatically published records should be treated as any other PROSPERO registration. Further detail is provided here.CitationHafiz Muhammad Ehsan Arshad, Muhammad Zain Raza, Muhammad Omais, Musab Maqsood, Muhammad Hashim Faisal, Ali Ahmad Nadeem. Comparative Analysis of Clinical Outcomes And Safety Profile of Penicillamine And Trientine in Management of Wilson’s disease: A Systematic Review