D-PENICILLAMINE AND ZINC THERAPY FOR WILSON’S DISEASE

March 1, 2011

V. Militaru 1, T. Pop 2, Lucia Burac 2, N. Miu 2, Ana Ştefănescu 2, Claudia Florentina Militaru 3, Ioana Corina Bocşan 3, Anca Buzoianu 3, C. Duncea 1
1 Medical Clinic V – “Iuliu Hatieganu” University of Medicine and Pharmacy, Cluj-Napoca
2 Pediatric Clinic II – “Iuliu Hatieganu” University of Medicine and Pharmacy, Cluj-Napoca
3 Department of Clinical Pharmacology – “Iuliu Hatieganu” University of Medicine and Pharmacy, Cluj-Napoca

Abstract

Wilson’disease (WD) is a rare genetic autosomal recessive condition characterized by Copper overload. Its treatment uses chelating agents (mainly D-Pencillamine) and drugs which lower the intestinal absorption of Copper (Zinc). We hypothesized that the drugs are efficient if they induce hepatic and/or neurological amelioration; the greater the efficiency, the shorter the period of time to the amelioration. On a group of 16 patients consulted or hospitalized in Cluj-Napoca (Romania) we compared the hepatic biochemical normalization and the neurological amelioration in the patients receiving Zinc monotherapy (7 patients) vs. association of Zinc and D-Penicillamine (9 patients). The results were in favor of the association of the two drugs, but the only parameter reaching statistical significance threshold was the time to hepatic and neurological amelioration (p = 0.023). There were no severe adverse effects of either drug. The small sample size might have reduced the chances to have more significant differences, issue to be addressed by further studies. We concluded that the association of D-Penicillamine and Zinc is safe and efficient in treating Wilson’s disease. Keywords: