Zinc acetate for the treatment of Wilson's disease.
Zinc acetate (Galzin, Gate Pharmaceutical Co.) has been developed for the treatment of Wilson's disease, an inherited disease of copper accumulation and copper toxicity in brain and liver. Zinc acetate has been approved by the US FDA for maintenance therapy of adult and paediatric Wilson's disease patients but also has efficacy in the treatment of pregnant patients and presymptomatic patients from the beginning. It also has value as adjunctive therapy for the initial treatment of symptomatic patients. Zinc's mechanism of action involves induction of intestinal cell metallothionein (Mt), which blocks copper absorption from the intestinal track. A negative copper balance is caused by blockade not only of absorption of food copper but the blockade of reabsorption of the considerable amount of endogenously secreted copper in saliva, gastric juice and intestinal secretions. Zinc is completely effective in controlling copper levels and toxicity in Wilson's disease, as are other anticopper agents. Zinc's major advantage over other anticopper agents is its extremely low level of toxicity. The only side effect is some degree of initial gastric irritation in approximately10% of patients, which usually decreases and becomes insignificant over time. As with all long-term therapies, compliance is a problem in some patients and dictates regular monitoring with 24 h urine copper and zinc measurements. As with all anticopper therapies, over a long period of time, overtreatment and induction of copper deficiency can occur. This is to be avoided particularly in children because copper is required for growth.
Brewer
G J
GJ
University of Michigan School of Medicine, Ann Arbor 48109-0618, USA. brewergj@umich.edu
D000818:Animals; D006527:Hepatolenticular Degeneration / Q000188:drug therapy* / Q000378:metabolism; D006801:Humans; D019345:Zinc Acetate / Q000493:pharmacokinetics / Q000627:therapeutic use*
D019345:Zinc Acetate