In a study appearing in this issue of HEPATOLOGY Brewer et al. present data on the results of pregnancies in 19 women treated with zinc acetate during 26 pregnancies, which resulted in 24 normal infants, 1 with a heart defect, and 1 microcephalic. Based on these results the authors conclude that ''zinc is the optimal choice for the pregnant Wilson' s disease patients,'' although no data on outcomes of pregnancies in women with Wilson' s disease treated with other agents are given.
Twenty-five years ago we reported on the outcomes of 29 pregnancies in 18 women treated with penicillamine giving birth to 29 normal infants. 2 Additional data were published by us 3 and by Walshe. Clearly, pregnancy in women with Wilson' s disease is safe and successful when treatment with a chelating drug is continued uninterruptedly. Since then, numerous reports regarding the outcomes of pregnancies in patients with Wilson' s disease confirm this statement (Table ).
Some of the therapeutic agents, namely dimercaprol, penicillamine, and trientine, chelate excess tissue copper, which is excreted via the urine. Zinc, in contrast, acts by inducing the synthesis of metallothionein, which sequesters copper in enterocytes and in hepatocytes. Unfortunately, no rigorously controlled, statistically valid study comparing the outcomes of various regimens has been performed and in all probability never will be. Penicillamine is the most widely used drug, frequently producing dramatic beneficial effects that enable many severely ill patients to return to normal, productive lives. Its long-term tolerance is demonstrated by patients who have remained asymptomatic by continuing to take their daily doses of penicillamine uninterruptedly for over 30 years! Trientine appears to be similarly effective based, however, on a more limited experience (Table ).
In the absence of controlled studies, we have pooled data concerning the results of pregnancy in women with Wilson' s disease receiving penicillamine or trientine: (1) Data of the National Center for the Study of Wilson' s Disease showed 26 women treated during 44 pregnancies in addition to the previously published cases, 2 for totals of 43 women treated with penicillamine during 71 pregnancies, and 4 women treated with trientine who delivered 6 normal babies. (2) Case reports from Medline showed 32 women treated with penicillamine and 2 with trientine during 43 pregnancies. (3) A personal communication from Dr. John M.