Abstract

IntroductionPregnancy management in women with Wilson disease (WD) remains an important clinical problem. This research was conducted to investigate how to avoid worsening of WD symptoms during pregnancy and increase pregnancy success in women with WD by identifying the best pregnancy management approaches in these patients.Patients and methodsThe clinical data of 117 pregnancies among 75 women with WD were retrospectively analyzed. Related information of the fetus was also recorded and analyzed. At the same time, regression analysis was performed for data of 22 pregnant women without WD, as normal controls.ResultsOf a total of 117 pregnancies among the 75 women with WD and 31 pregnancies among the 22 control womenincluded in this study, there were 108 successful pregnancies and 9 spontaneous abortions. Among the 108 successful pregnancies, 97 women a history of copper chelation therapy before pregnancy; all 97 women stopped anti-copper therapy during pregnancy. The nine women with spontaneous abortion had no pre-pregnancy history of copper displacement therapy. The incidence of lower limb edema was higher in the WD group than in normal controls (P = 0.036). Compared with the control group, there was a higher proportion in the WD group of male infants (P = 0.022) and lower average infant birth weight (t = 3.514, P = 0.001).ConclusionIt 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.

Highlights

  • Pregnancy management in women with Wilson disease (WD) remains an important clinical problem

  • Of a total of 117 pregnancies among the 75 women with WD and 31 pregnancies among the 22 control womenincluded in this study, there were 108 successful pregnancies and 9 spontaneous abortions

  • Statistical methods Quantitative data consistent with a normal distribution are described as mean ± standard deviation (SD), and the pregnancy status of women in the WD and control groups was compared using a t-test of two independent samples

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Summary

Introduction

Pregnancy management in women with Wilson disease (WD) remains an important clinical problem. This research was conducted to investigate how to avoid worsening of WD symptoms during pregnancy and increase pregnancy success in women with WD by identifying the best pregnancy management approaches in these patients. Wilson disease (WD) is an autosomal recessive genetic disorder that can be treated at present [1]. WD is caused by excessive deposition of ATP7B copper transporter protein, which causes a variety of symptoms in different organs [2]. The most common copper deposition sites are the liver and the brain [1]. Few studies have reported on pregnancy in patients with WD, and the findings of the existing research vary considerably.

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