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A B Taly - One of the best experts on this subject based on the ideXlab platform.
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withdrawal of Penicillamine from zinc sulphate Penicillamine maintenance therapy in wilson s disease promising safe and cheap
Journal of the Neurological Sciences, 2008Co-Authors: S Sinha, A B TalyAbstract:Abstract Background Penicillamine, once considered the cornerstone of treatment for Wilson disease (WD), is rather expensive and toxic, and often causes neurological worsening. Zinc sulphate, aiming at the treatment of free-copper toxicosis, has emerged as effective, safe and cheap alternative. Aim To assess the effect of withdrawal of Penicillamine from maintenance treatment with Penicillamine and zinc sulphate. Patients and methods 45 patients of WD (M:F: 28:17; age at diagnosis: 13.5 ± 63 years), on both Penicillamine (P) and zinc sulphate (Zn), couldn’t continue Penicillamine due to financial constraints. Their clinical data, disability and impairment scores (Schwab and England (SE p = 0.4), NSS (1.8 ± 3.1 vs. 1.5 ± 2.3; p = 0.03) and SE p = 0.03). There were no adverse effects. Conclusions Withdrawal of Penicillamine from zinc sulphate/Penicillamine maintenance therapy for patients with Wilson's disease was effective, safe and economic, for almost all patients. This retrospective study reiterates that zinc sulphate may be used as a preferred mode of treatment for patients with Wilson's disease.
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Withdrawal of Penicillamine from zinc sulphate-Penicillamine maintenance therapy in Wilson's disease: promising, safe and cheap.
Journal of the neurological sciences, 2007Co-Authors: S Sinha, A B TalyAbstract:Penicillamine, once considered the cornerstone of treatment for Wilson disease (WD), is rather expensive and toxic, and often causes neurological worsening. Zinc sulphate, aiming at the treatment of free-copper toxicosis, has emerged as effective, safe and cheap alternative. To assess the effect of withdrawal of Penicillamine from maintenance treatment with Penicillamine and zinc sulphate. 45 patients of WD (M:F: 28:17; age at diagnosis: 13.5+/-63 years), on both Penicillamine (P) and zinc sulphate (Zn), couldn't continue Penicillamine due to financial constraints. Their clinical data, disability and impairment scores (Schwab and England (S&E) score, Neurological Symptom Score (NSS), and Chu staging) and follow-up data of patients maintained only on zinc sulphate were recorded. Majority of patients (84.4%) had neuropsychiatric manifestations. The mean duration of treatment with Penicillamine (P) and zinc sulphate (P+Zn), before stopping Penicillamine, was 107.4+/-67.3 months. 40 patients improved variably, while the rest didn't. They received only zinc sulphate for 27.2+/-8.5 months (range: 12 to 34) and 44 patients (97.7%) remained status quo or improved marginally. Only one patient reported worsening in dysarthria. Their disability and impairment scores during combination (Penicillamine and zinc sulphate) and Zn alone were: Chu (1.3+/-0.5 vs. 1.5+/-1.9; p=0.4), NSS (1.8+/-3.1 vs. 1.5+/-2.3; p=0.03) and S&E (96.4+/-5.6 vs. 98.6+/-3.5; p=0.03). There were no adverse effects. Withdrawal of Penicillamine from zinc sulphate/Penicillamine maintenance therapy for patients with Wilson's disease was effective, safe and economic, for almost all patients. This retrospective study reiterates that zinc sulphate may be used as a preferred mode of treatment for patients with Wilson's disease.
S Sinha - One of the best experts on this subject based on the ideXlab platform.
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withdrawal of Penicillamine from zinc sulphate Penicillamine maintenance therapy in wilson s disease promising safe and cheap
Journal of the Neurological Sciences, 2008Co-Authors: S Sinha, A B TalyAbstract:Abstract Background Penicillamine, once considered the cornerstone of treatment for Wilson disease (WD), is rather expensive and toxic, and often causes neurological worsening. Zinc sulphate, aiming at the treatment of free-copper toxicosis, has emerged as effective, safe and cheap alternative. Aim To assess the effect of withdrawal of Penicillamine from maintenance treatment with Penicillamine and zinc sulphate. Patients and methods 45 patients of WD (M:F: 28:17; age at diagnosis: 13.5 ± 63 years), on both Penicillamine (P) and zinc sulphate (Zn), couldn’t continue Penicillamine due to financial constraints. Their clinical data, disability and impairment scores (Schwab and England (SE p = 0.4), NSS (1.8 ± 3.1 vs. 1.5 ± 2.3; p = 0.03) and SE p = 0.03). There were no adverse effects. Conclusions Withdrawal of Penicillamine from zinc sulphate/Penicillamine maintenance therapy for patients with Wilson's disease was effective, safe and economic, for almost all patients. This retrospective study reiterates that zinc sulphate may be used as a preferred mode of treatment for patients with Wilson's disease.
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Withdrawal of Penicillamine from zinc sulphate-Penicillamine maintenance therapy in Wilson's disease: promising, safe and cheap.
Journal of the neurological sciences, 2007Co-Authors: S Sinha, A B TalyAbstract:Penicillamine, once considered the cornerstone of treatment for Wilson disease (WD), is rather expensive and toxic, and often causes neurological worsening. Zinc sulphate, aiming at the treatment of free-copper toxicosis, has emerged as effective, safe and cheap alternative. To assess the effect of withdrawal of Penicillamine from maintenance treatment with Penicillamine and zinc sulphate. 45 patients of WD (M:F: 28:17; age at diagnosis: 13.5+/-63 years), on both Penicillamine (P) and zinc sulphate (Zn), couldn't continue Penicillamine due to financial constraints. Their clinical data, disability and impairment scores (Schwab and England (S&E) score, Neurological Symptom Score (NSS), and Chu staging) and follow-up data of patients maintained only on zinc sulphate were recorded. Majority of patients (84.4%) had neuropsychiatric manifestations. The mean duration of treatment with Penicillamine (P) and zinc sulphate (P+Zn), before stopping Penicillamine, was 107.4+/-67.3 months. 40 patients improved variably, while the rest didn't. They received only zinc sulphate for 27.2+/-8.5 months (range: 12 to 34) and 44 patients (97.7%) remained status quo or improved marginally. Only one patient reported worsening in dysarthria. Their disability and impairment scores during combination (Penicillamine and zinc sulphate) and Zn alone were: Chu (1.3+/-0.5 vs. 1.5+/-1.9; p=0.4), NSS (1.8+/-3.1 vs. 1.5+/-2.3; p=0.03) and S&E (96.4+/-5.6 vs. 98.6+/-3.5; p=0.03). There were no adverse effects. Withdrawal of Penicillamine from zinc sulphate/Penicillamine maintenance therapy for patients with Wilson's disease was effective, safe and economic, for almost all patients. This retrospective study reiterates that zinc sulphate may be used as a preferred mode of treatment for patients with Wilson's disease.
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Withdrawal of Penicillamine from zinc sulphate–Penicillamine maintenance therapy in Wilson's disease: Promising, safe and cheap
Journal of the Neurological Sciences, 2007Co-Authors: S Sinha, TalyAbstract:Abstract Background Penicillamine, once considered the cornerstone of treatment for Wilson disease (WD), is rather expensive and toxic, and often causes neurological worsening. Zinc sulphate, aiming at the treatment of free-copper toxicosis, has emerged as effective, safe and cheap alternative. Aim To assess the effect of withdrawal of Penicillamine from maintenance treatment with Penicillamine and zinc sulphate. Patients and methods 45 patients of WD (M:F: 28:17; age at diagnosis: 13.5 ± 63 years), on both Penicillamine (P) and zinc sulphate (Zn), couldn’t continue Penicillamine due to financial constraints. Their clinical data, disability and impairment scores (Schwab and England (SE p = 0.4), NSS (1.8 ± 3.1 vs. 1.5 ± 2.3; p = 0.03) and SE p = 0.03). There were no adverse effects. Conclusions Withdrawal of Penicillamine from zinc sulphate/Penicillamine maintenance therapy for patients with Wilson's disease was effective, safe and economic, for almost all patients. This retrospective study reiterates that zinc sulphate may be used as a preferred mode of treatment for patients with Wilson's disease.
Taly - One of the best experts on this subject based on the ideXlab platform.
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Withdrawal of Penicillamine from zinc sulphate–Penicillamine maintenance therapy in Wilson's disease: Promising, safe and cheap
Journal of the Neurological Sciences, 2007Co-Authors: S Sinha, TalyAbstract:Abstract Background Penicillamine, once considered the cornerstone of treatment for Wilson disease (WD), is rather expensive and toxic, and often causes neurological worsening. Zinc sulphate, aiming at the treatment of free-copper toxicosis, has emerged as effective, safe and cheap alternative. Aim To assess the effect of withdrawal of Penicillamine from maintenance treatment with Penicillamine and zinc sulphate. Patients and methods 45 patients of WD (M:F: 28:17; age at diagnosis: 13.5 ± 63 years), on both Penicillamine (P) and zinc sulphate (Zn), couldn’t continue Penicillamine due to financial constraints. Their clinical data, disability and impairment scores (Schwab and England (SE p = 0.4), NSS (1.8 ± 3.1 vs. 1.5 ± 2.3; p = 0.03) and SE p = 0.03). There were no adverse effects. Conclusions Withdrawal of Penicillamine from zinc sulphate/Penicillamine maintenance therapy for patients with Wilson's disease was effective, safe and economic, for almost all patients. This retrospective study reiterates that zinc sulphate may be used as a preferred mode of treatment for patients with Wilson's disease.
Carol Baker - One of the best experts on this subject based on the ideXlab platform.
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D-Penicillamine in systemic sclerosis? Yes!
Scandinavian journal of rheumatology, 2001Co-Authors: Thomas A. Medsger, Mary Lucas, Kathryn S. Wildy, Carol BakerAbstract:The use of D-Penicillamine in systemic sclerosis (SSc) has been controversial. We have reviewed the major published studies on this drug in SSc with diffuse cutaneous (dc) involvement and summarized our own recent experience in dcSSc patients treated with and without D-Penicillamine. We conclude that D-Penicillamine favourably alters the natural history of skin involvement in dcSSc, even when used in low dose. Furthermore, recurrence of diffuse skin change after discontinuation of D-Penicillamine and improvement in skin thickening after reinitiation of the drug support its effectiveness. We believe that the rheumatologic community should use D-Penicillamine in patients with early dcSSc.
Andreas Papapetropoulos - One of the best experts on this subject based on the ideXlab platform.
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d Penicillamine modulates hydrogen sulfide h2s pathway through selective inhibition of cystathionine γ lyase
British Journal of Pharmacology, 2016Co-Authors: Vincenzo Brancaleone, Iolanda Esposito, Antonella Gargiulo, Valentina Vellecco, Antonia Asimakopoulou, Valentina Citi, Vincenzo Calderone, Thomas Gobbetti, Mauro Perretti, Andreas PapapetropoulosAbstract:BACKGROUND AND PURPOSE Hydrogen sulfide (H2S) is a gasotransmitter produced from L-cysteine through the enzymatic action of cystathionine-γ-lyase (CSE) and/or cystathionine-β-synthase. D-Penicillamine is the d isomer of a dimethylated cysteine and has been used for the treatment of rheumatoid arthritis. AsD-Penicillamine is structurally very similar to cysteine, we have investigated whether D-Penicillamine, as a cysteine analogue, has an effect on the H2 S pathway. EXPERIMENTAL APPROACH We tested the effect of D-Penicillamine (0.01-1 mM) in mouse aortic rings mounted in isolated organ baths and determined whether it could affect H2 S biosynthesis. In particular, we investigated any possible inhibitor or donor behaviour by using recombinant enzyme-based assays and an in vivo approach. KEY RESULTS D-Penicillamine, per se, showed little or no vasodilator effect, and it cannot be metabolized as a substrate in place of l-cysteine. However, d-Penicillamine significantly reduced L-cysteine-induced vasodilatation in a concentration-dependent manner through inhibition of H2 S biosynthesis, and this effect occurred at concentrations 10 times lower than those needed to induce the release of H2 S. In particular, D-Penicillamine selectively inhibited CSE in a pyridoxal-5'-phosphate-dependent manner. CONCLUSIONS AND IMPLICATIONS Taken together, our results suggest that D-Penicillamine acts as a selective CSE inhibitor, leading to new perspectives in the design and use of specific pharmacological tools for H2 S research. In addition, the inhibitory effect of D-Penicillamine on CSE could account for its beneficial action in rheumatoid arthritis patients, where H2 S has been shown to have a detrimental effect.