Zinc therapy for Wilson's disease: study of 20 cases

ZINC THERAPY FOR WILSON'S DISEASE: STUDY OF 20 CASES (ABSTRACT)^*. THESIS. SÃO PAULO, 2000.

EGBERTO REIS BARBOSA^**

Eleven female and nine male subjects with Wilson's disease (WD) were included in this study. Data collection ran from September 1983 until December 1999. All subjects were submitted to zinc therapy during the study follow-up.

Patients were split into two groups. Group 1 included the subjects who had been on adequate zinc therapy for over a year. Subsequently, group 1 was further subdivided into 1-A and 1-B, to include subjects who had been on D-Penicillamine (DP) for under and over five years, respectively. Group 2 was also subdivided into 2-A, with subjects who had received zinc plus DP, and group 2-B, with patients showing an advanced stage of the disease.

Zinc therapy outcomes were assessed considering the evolution of neurological and systemic clinical pictures, Kayser-Fleischer (K-F) rings, liver function blood tests, serum and urine copper levels and, in several cases, Magnetic Resonance Imaging (MRI) of the head. The neurological clinical work-up and MRI results were quantified and pre and post treatment scores developed.

The mean age of disease onset was 18.5 ± 13.2 years and the mean present age of subjects was 33.3 ± 13.2 years. Of the eighteen patients presenting the neurological form of the disease, one had the hepatic form whilst another had the hematological form of WD. All 20 subjects, except one, were treated initially with DP, where the causes that most frequently determined its substitution by zinc, were leukopenia (8 cases) and kidney disease (5 cases). Group 1 average treatment duration with DP was 7.9 years, whereas the average treatment duration with zinc was 4.8 years.

On comparison, compliance rate averages for zinc and DP therapy indicated a trend toward a greater treatment compliance rate with zinc therapy. Averaged neurological scores before and after zinc treatment showed an improvement in the neurological profile, with score reduction following zinc therapy in group 1. Out of 20 cases, eight subjects presented a total or partial regression of K-F rings, 12 cases remained unchanged and only one relapsed. Out of the seven patients who were submitted to a head MRI, four showed an improvement in signal abnormalities, two remained unchanged and one presented increased MRI changes.

The overall treatment outcome showed that zinc therapy was effective (leading to an improved or stable clinical picture) in 10 out of the 17 patients (58.8 %) who had received zinc in an appropriate fashion. In group1, Zinc therapy was effective in 10 out 15 cases (66.7 %) where compliance rate was good in 86.7 % of patients evaluated. These results do not seem influenced by previous treatment with DP given no statistical relationship between DP treatment duration and good response to zinc therapy was observed. The sole side-effect of zinc salts seen in any of the 20 patients evaluated, was epigastralgia, recorded in six subjects (30 %).

The results obtained in this study indicate that zinc is an adequate therapeutic alternative in treating WD where DP needs to be substituted.

KEY WORDS: Wilson's disease, zinc therapy.

**Address: Divisão de Neurologia do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, Avenida Enéas Carvalho Aguiar 255, 05403-000 São Paulo SP, Brasil. E-mail: [e-mail]

*

O zinco no tratamento da doença de Wilson: estudo de de 20 casos (Resumo). Tese de Livre-Docência, Faculdade de Medicina da Universidade de São Paulo (Disciplina de Neurologia Clínica).

Publication Dates

• Publication in this collection
  05 Oct 2001
• Date of issue
  Sept 2001