Withdrawal of penicillamine from zinc sulphate-penicillamine maintenance therapy in Wilson's disease: promising, safe and cheap.
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.


OBJECTIVE
To assess the effect of withdrawal of penicillamine from maintenance treatment with penicillamine and zinc sulphate.


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 (S&E) score, Neurological Symptom Score (NSS), and Chu staging) and follow-up data of patients maintained only on zinc sulphate were recorded.


RESULTS
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.


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.
Sinha
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National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore - 560 029, India. sanjib_sinha2004@yahoo.co.in
Taly
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D000293:Adolescent; D000328:Adult; D001252:Astringents / Q000008:administration & dosage / Q000191:economics; D002614:Chelating Agents / Q000008:administration & dosage / Q000009:adverse effects / Q000191:economics; D015913:Chelation Therapy / Q000009:adverse effects / Q000191:economics / Q000379:methods; D002648:Child; D002675:Child, Preschool; D003300:Copper / Q000378:metabolism / Q000633:toxicity; D005260:Female; D006527:Hepatolenticular Degeneration / Q000188:drug therapy* / Q000378:metabolism / Q000503:physiopathology; D006801:Humans; D008297:Male; D019965:Neurocognitive Disorders / Q000139:chemically induced / Q000378:metabolism / Q000503:physiopathology; D010396:Penicillamine / Q000008:administration & dosage* / Q000009:adverse effects / Q000191:economics; D012189:Retrospective Studies; D016896:Treatment Outcome; D019287:Zinc Sulfate / Q000008:administration & dosage* / Q000191:economics
D001252:Astringents; D002614:Chelating Agents; D019287:Zinc Sulfate; D003300:Copper; D010396:Penicillamine