Pregnancy outcome after chelation therapy in Wilson disease. Evaluation of the German Embryotox Database

- Pregnant women with Wilson disease should continue chelation therapy.
- Dose reduction of chelators about 25-50% should be considered in pregnancy.
- Our case series did not show major birth defects after maternal chelation exposure.
- The teratogenic risk seems to be very low after well controlled maternal chelator therapy.

Continuation of treatment is recommended for pregnant women with Wilson disease. Therapy options include the copper chelating agents d-penicillamine and trientine. However, there are still uncertainties concerning a possible teratogenic risk. In this case series, we report on the outcome of 20 pregnancies with maternal chelator exposure at least during the first trimester. Of these 20 pregnancies documented by the German Embryotox Project, 14 were prospectively ascertained and 6 were retrospective. No major birth defects were observed. Three of the 14 prospective cases resulted in a spontaneous abortion, and one pregnancy was electively terminated. Our results do not support the hypothesis of teratogenicity based on earlier case reports of congenital anomalies. Therefore our study may contribute to reassure women needing chelation therapy during pregnancy. However, it must be taken into account that the sample size of this case series is too limited to make final conclusions on teratogenic effects.