The Experts below are selected from a list of 102 Experts worldwide ranked by ideXlab platform
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.
Russell J. Mumper - One of the best experts on this subject based on the ideXlab platform.
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An investigation into copper catalyzed D-Penicillamine oxidation and subsequent hydrogen peroxide generation.
Journal of inorganic biochemistry, 2006Co-Authors: Anshul Gupte, Russell J. MumperAbstract:D-Penicillamine is a potent copper (Cu) chelating agent. D-Pen reduces Cu(II) to Cu(I) in the process of chelation while at the same time being oxidized to D-Penicillamine disulfide. It has been proposed that hydrogen peroxide is generated during this process. However, definitive experimental proof that hydrogen peroxide is generated remains lacking. Thus, the major aims of these studies were to confirm and quantitatively assess the in vitro production of hydrogen peroxide during copper catalyzed D-Penicillamine oxidation. The potential cytotoxic effect of hydrogen peroxide generation was also investigated in vitro against MCF-7 human breast cancer cells. Cell cytotoxicity resulting from the incubation of D-Penicillamine with copper was compared to that of D-Penicillamine, copper and hydrogen peroxide. The mechanism of copper catalyzed D-Penicillamine oxidation and simultaneous hydrogen peroxide production was investigated as a function of time, concentration of cupric sulfate or ferric chloride, temperature, pH, anaerobic condition and chelators such as ethylenediaminetetraacetic acid and bathocuproinedisulfonic acid. A simple, sensitive and rapid HPLC assay was developed to simultaneously detect D-Penicillamine, its major oxidation product D-Penicillamine disulfide, and hydrogen peroxide in a single run. Hydrogen peroxide was shown to be generated in a concentration dependent manner as a result of D-Penicillamine oxidation in the presence of cupric sulfate. Chelators such as ethylenediaminetetraacetic acid and bathocuproinedisulfonic acid were able to inhibit D-Penicillamine oxidation. The incubation of MCF-7 human breast cancer cells with D-Penicillamine plus cupric sulfate resulted in the production of reactive oxygen species within the cell and cytotoxicity that was comparable to free hydrogen peroxide.
Juan I. Arenas - One of the best experts on this subject based on the ideXlab platform.
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Is D-Penicillamine useful in fulminat Wilson's disease?
Liver Transplantation, 2002Co-Authors: Jorge Rakela, Hugo E. Vargas, Juan I. ArenasAbstract:Background: Wilson's disease, heralded by severe hepatic insufficiency, is a rare disorder for which emergency liver transplantation is considered to be the only effective therapy. Aims: To report the features of Wilson's disease with severe hepatic insufficiency in a series of 17 patients and, during the second period of the study, to assess the efficacy of a policy consisting of early administration of D-Penicillamine. Patients: Seventeen consecutive patients with Wilson's disease were studied. During the first period of the study (up to 1979), none of the patients received D-Penicillamine. During the second period (after 1979), all patients without encephalopathy at admission received D-Penicillamine. Results: The four patients observed during the first period who did not have encephalopathy at admission and did not receive D-Penicillamine progressed to encephalopathy and died. Among the 13 consecutive patients observed during the second period, two patients with encephalopathy at admission did not receive D-Penicillamine and were transplanted. The 11 remaining patients all received D-Penicillamine. Ten of these patients survived without the need for transplantation and returned to compensated liver disease without liver insufficiency. In one patient, liver insufficiency progressed and transplantation had to be performed. Conclusions: In most patients with Wilson's disease heralded by severe hepatic insufficiency and without encephalopathy at admission, early administration of D-Penicillamine was associated with survival without transplantation. These results suggest the importance of early diagnosis of this form of Wilson's disease before the onset of encephalopathy, and favour early administration of D-Penicillamine which could avoid the need for transplantation in most cases.
