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. 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 (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.

Aluminum chloride hexahydrate versus palmar hyperhidrosis. Evaporimeter assessment.

The efficacy of topical aluminium chloride hexahydrate 20% W/W ethanol (ACH) in the treatment symptomatic palmar hyperhidrosis was studied in 12 patients. A half-sided control single blind (assessor blind) study was done. Patients applied ACH on one palm daily for 4 weeks. The response to treatment was measured objectively with an evaporimeter. There was significant fall of skin water vapor loss (SVL) on treated palms compared with untreated palms. The basal mean SVL of treated palms and untreated palms were 79.9 and 77.9 g water/m2/h, respectively (n.s.). The mean SVLs of treated vs. untreated palms at week 1, 2, 3, and 4 were 66.4 vs. 79.7 (p less than 0.05), 56.6 vs. 72.2 (p less than 0.001), 58.2 vs. 72.5 (p = 0.1), and 51.4 vs. 72.7 (p less than 0.001) g water/m2/h, respectively. The mean SVL of treated palms returned near basal rate within 1 week of stopping treatment. Four patients developed skin irritation from ACH; in three this disappeared after 1 week and they were able to continue with treatment; one withdrew from the study because of the severe irritation. All patients reported that the ACH reduced palmar sweating within 48 hours of application; its effect disappeared within 48 hours after stopping treatment. ACH appeared to be useful in rapid control of palmar hyperhydrosis.