WD is an autosomal recessive hereditary disease that can be treated at present(3–4). Many patients with WD disease are women of childbearing age who are diagnosed with early-stage disease(17,18). Some women with WD become pregnant after disease onset and diagnosis; therefore, further research on the management of pregnant patients with WD is urgently needed. This retrospective analysis was conducted, using data of pregnant women with WD in China, focusing on several aspects and comparing choices in pregnancy, to better guide women of childbearing age who have WD.
In the present retrospective analysis of 117 pregnancies in 75 women with WD, there were 108 successful pregnancies (92.3%) and successful deliveries. The vast majority of the patients included in this study had a history of pre-pregnancy hospitalization in which they received treatment with copper chelation therapy and disease evaluation during the first half of their pregnancy. These results are not completely consistent with the literature(15,19). Therefore, it is strongly recommended that women with WD need systematic evaluation and treatment during pre-pregnancy.Studies have found that the clinical effects of WD on pregnancy outcomes mainly include neurological symptoms in pregnant women, as well as spontaneous abortion (7–8,19). The data analyzed in the present study showed that neurological symptoms were more frequent among women with WD than spontaneous abortions or liver and bone damage. These findings are consistent with the results of the present analysis, in which all women with spontaneous abortion had no prior history of anti-copper therapy.
Analysis of the present data showed that hospitalized women with WD received copper displacement treatment and disease evaluation before their pregnancy; these patients stopped taking copper displacement medications during pregnancy. This finding is inconsistent with reports in the recent literature (13–14); additional multicenter studies, are needed to clarify this issue. The authors believe that clinical symptoms in these patients are relatively reduced in pregnancy, which could be related to normal metabolism of copper by the fetus. This result also found that patients with hospital readmission after childbirth for symptoms of liver injury gradually recovered after copper chelation treatment. However, recovery was slower after anti-copper treatment in patients readmitted to the hospital after childbirth with aggravated neurological symptoms. These findings are in line with those of recent reports (7,19).The aggravation of neurological symptoms in women with WD during pregnancy has been widely reported (6–7,19), but the specific mechanism has not been further explored. The present data analysis indicated that neurological symptoms were aggravated in 10 pregnant women with WD, and 24-hour urine monitoring showed that copper levels were not very high. Therefore, aggravation of neurological symptoms in patients with WD may not be completely consistent with excessive copper deposition in the body, which differs considerably from published reports (6–7,19).
Many researchers have stated that the most important factor influencing the pregnancy outcome of women with WD is continuous copper displacement therapy, and that continuous treatment is the best approach to avoid aggravation of the disease and increase the success rate of pregnancy (10–15,19). The authors believe that the pre-pregnancy copper displacement treatment and condition assessment, followed by suspension of drug therapy during pregnancy is the best way to avoid aggravated disease symptoms during pregnancy and to increase the success rate of pregnancy in women with WD.
In our study population, women with WD were more likely to have complications of lower limb edema during pregnancy than normal pregnant women, and more women in the WD group had male infants and infants with lower birth weight than women in the control group. However, in terms of other pregnancy complications and complications, there was no difference in the mode of delivery or fetal Apgar score between the patient groups. Whether our findings can comprehensively reflect the pregnancy status of women with WD requires confirmation in future studies.
To sum up, women with WD have complicated issues in pregnancy, and prospective studies in this patient population are lacking; all published reports are retrospective analyses. It is relatively safe for women with WD patients to become pregnant. The best management method for pregnancy in women with WD may be intensive pre-pregnancy copper chelation therapy and no anti-copper treatment during pregnancy.