Efficacy of initial chelation with penicillamine followed by zinc monotherapy in symptomatic Wilson's disease.

Mehul, Choksi (2010) Efficacy of initial chelation with penicillamine followed by zinc monotherapy in symptomatic Wilson's disease. Masters thesis, Christian Medical College, Vellore.


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INTRODUCTION : Wilson’s disease (WD) is an autosomal recessive disorder of copper metabolism which is caused by failure of biliary excretion of excess copper. The excess copper toxicity results in damage to the organs, primarily brain and/or liver 1. This disorder is progressive and ultimately fatal if not recognized and treated. OBJECTIVES: There are no recommendations for use of zinc in symptomatic Wilson’s disease (WD). The aim was to study the clinical outcome of patients with symptomatic WD who were changed from penicillamine to zinc due to financial constraints or adverse effects. METHODS: All patients of patients of symptomatic hepatic +/- neurological/psychiatric manifestations of WD changed over from penicillamine to zinc sulfate and formed the study cohort. Diagnosis other than WD, atypical copper deposition disease and presymptomatic WD were excluded. Clinical and laboratory details at presentation, on penicillamine and on zinc were recorded. At each follow up visit and clinical worsening or improvement, compliance and adverse effects were recorded. The patients had periodic monitoring of hemoglobin, TC/DC, platelet count, liver function tests and prothrombin time. MELD and Nazer score and NWI were calculated at presentation, on penicillamine and on zinc. ANOVA was the statistical test used for comparing the means of variables at baseline, on penicillamine and on zinc therapy. RESULTS: 28 patients formed the study cohort. 26 with pure hepatic manifestation, 2 with added neurological and 2 with added psychiatric manifestations. The mean age of subjects was 17.5 ± 6.19 years. Mean duration of penicillamine therapy was 123.92 weeks (SD: 94.18; range: 2-320 weeks). The mean duration of zinc therapy was 175.92 weeks (SD: 122.82; range: 21 to 352 weeks). On follow up there was serial improvement in the serum bilirubin, serum albumin, PT INR, MELD score, Nazer score and Wilson disease prognostic index. Penicillamine is a good drug for initial chelation therapy for patients with symptomatic WD. Once switched over to zinc monotherapy these patients continue to remain compensated with maintenance or improvement of laboratory parameters and/or clinical scores. Penicillamine has serious and life threatening side effects where as zinc has minimal, tolerable and non life threatening side effects. Initial chelation with penicillamine followed by maintenance zinc monotherapy is an efficacious regime to treat symptomatic patients of hepatic Wilson’s disease.

Item Type: Thesis (Masters)
Uncontrolled Keywords: Efficacy ; initial chelation ; penicillamine ; zinc ; monotherapy ; symptomatic ; Wilson's disease.
Subjects: MEDICAL > Gastroenterology
Depositing User: Kambaraman B
Date Deposited: 14 Jul 2017 04:43
Last Modified: 14 Jul 2017 04:43
URI: http://repository-tnmgrmu.ac.in/id/eprint/1632

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