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Published in: BMC Pregnancy and Childbirth 1/2019

Open Access 01-12-2019 | Wilson's Disease | Research article

The study of Wilson disease in pregnancy management

Authors: Xu-En Yu, Min Pan, Yong-Zhu Han, Ren-Min Yang, Juan Wang, Shan Gao

Published in: BMC Pregnancy and Childbirth | Issue 1/2019

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Abstract

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.

Patients and methods

The 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.

Results

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. 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).

Conclusion

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.
Literature
1.
go back to reference Bandmann O, Weiss KH, Kaler SG. Wilson’s disease and other neurological copper disorders. Lancet Neurol. 2015;14:103–13.CrossRef Bandmann O, Weiss KH, Kaler SG. Wilson’s disease and other neurological copper disorders. Lancet Neurol. 2015;14:103–13.CrossRef
2.
go back to reference Weiss KH, Stremmel W. Clinical considerations for an effective medical therapy in Wilson's disease. Ann N Y Acad Sci. 2014;1315:81–5.CrossRef Weiss KH, Stremmel W. Clinical considerations for an effective medical therapy in Wilson's disease. Ann N Y Acad Sci. 2014;1315:81–5.CrossRef
3.
go back to reference European Association for Study of Liver. EASL clinical practice guidelines: Wilson’s disease. J Hepatol. 2012;56:671–85.CrossRef European Association for Study of Liver. EASL clinical practice guidelines: Wilson’s disease. J Hepatol. 2012;56:671–85.CrossRef
4.
go back to reference Roberts EA, Schilsky ML. Diagnosis and treatment of Wilson disease: an update. Hepatology. 2008;47:2089–111.CrossRef Roberts EA, Schilsky ML. Diagnosis and treatment of Wilson disease: an update. Hepatology. 2008;47:2089–111.CrossRef
5.
go back to reference Katarina D, Evelin B, Christof S. Pregnancy outcome after chelation therapy in Wilson disease. Evaluation of the German Embryotox database. Reprod Toxicol. 2016;65:39–45.CrossRef Katarina D, Evelin B, Christof S. Pregnancy outcome after chelation therapy in Wilson disease. Evaluation of the German Embryotox database. Reprod Toxicol. 2016;65:39–45.CrossRef
6.
go back to reference Furman B, Bashiri A, Wiznitzer A, et al. Wilson’s disease in pregnancy: five successful consecutive pregnancies of the same woman. Eur J Obstet Gynecol Reprod Biol. 2001;96:232–4.CrossRef Furman B, Bashiri A, Wiznitzer A, et al. Wilson’s disease in pregnancy: five successful consecutive pregnancies of the same woman. Eur J Obstet Gynecol Reprod Biol. 2001;96:232–4.CrossRef
7.
go back to reference Zegarac Z, Duić Z, Stasenko S, et al. Wilson’s disease in pregnancy. Acta Clin Croat. 2013;52:529–32.PubMed Zegarac Z, Duić Z, Stasenko S, et al. Wilson’s disease in pregnancy. Acta Clin Croat. 2013;52:529–32.PubMed
8.
go back to reference Shimono N, Ishibashi H, Ikematsu H, et al. Fulminant hepatic failure during perinatal period in a pregnant woman with Wilson's disease. Gastroenterol Jpn. 1991;26:69–73.CrossRef Shimono N, Ishibashi H, Ikematsu H, et al. Fulminant hepatic failure during perinatal period in a pregnant woman with Wilson's disease. Gastroenterol Jpn. 1991;26:69–73.CrossRef
9.
go back to reference Yu XE, Gao S, Yang RM, et al. MR imaging of the brain in neurologic Wilson disease. AJNR Am J Neuroradiol. 2019;40:178–83.CrossRef Yu XE, Gao S, Yang RM, et al. MR imaging of the brain in neurologic Wilson disease. AJNR Am J Neuroradiol. 2019;40:178–83.CrossRef
10.
go back to reference Berghella V, Steele D, Spector T, et al. Successful pregnancy in a neurologically impaired woman with Wilson's disease. Am J Obstet Gynecol. 1997;176:712–4.CrossRef Berghella V, Steele D, Spector T, et al. Successful pregnancy in a neurologically impaired woman with Wilson's disease. Am J Obstet Gynecol. 1997;176:712–4.CrossRef
11.
go back to reference Devesa R, Alvarez A, de las Heras G, et al. Wilson’s disease treated with trientine during pregnancy. J Pediatr Gastroenterol Nutr. 1995;20:102–3.CrossRef Devesa R, Alvarez A, de las Heras G, et al. Wilson’s disease treated with trientine during pregnancy. J Pediatr Gastroenterol Nutr. 1995;20:102–3.CrossRef
12.
go back to reference Dreifuss FE, McKinney WM. Wilson’s disease (hepatolenticular degeneration) and pregnancy. JAMA. 1966;195:960–2.CrossRef Dreifuss FE, McKinney WM. Wilson’s disease (hepatolenticular degeneration) and pregnancy. JAMA. 1966;195:960–2.CrossRef
13.
go back to reference Lee HJ, Seong WJ, Hong SY, et al. Successful pregnancy outcome in a Korean patient with symptomatic Wilson's disease. Obstet Gynecol Sci. 2015;58:409–13.CrossRef Lee HJ, Seong WJ, Hong SY, et al. Successful pregnancy outcome in a Korean patient with symptomatic Wilson's disease. Obstet Gynecol Sci. 2015;58:409–13.CrossRef
14.
go back to reference Scheinberg IH, Sternlieb I. Pregnancy in penicillamine-treated patients with Wilson's disease. N Engl J Med. 1975;293:1300–2.CrossRef Scheinberg IH, Sternlieb I. Pregnancy in penicillamine-treated patients with Wilson's disease. N Engl J Med. 1975;293:1300–2.CrossRef
15.
go back to reference Walshe JM. Pregnancy in Wilson’s disease. Q J Med. 1977;46:73–83.PubMed Walshe JM. Pregnancy in Wilson’s disease. Q J Med. 1977;46:73–83.PubMed
16.
go back to reference Rabiee A, Hamilton JPA. Pregnancy in Wilson disease. Hepatology. 2018;67:1201–3.CrossRef Rabiee A, Hamilton JPA. Pregnancy in Wilson disease. Hepatology. 2018;67:1201–3.CrossRef
17.
go back to reference Yang RM. Hepatolenticular degeneration. Beijing: People’s Medical Publishing House; 2015. p. 427–9. Yang RM. Hepatolenticular degeneration. Beijing: People’s Medical Publishing House; 2015. p. 427–9.
18.
go back to reference Schilsky ML. Wilson disease diagnosis, treatment, and follow-up. Clin Liver Dis. 2017;21:755–67.CrossRef Schilsky ML. Wilson disease diagnosis, treatment, and follow-up. Clin Liver Dis. 2017;21:755–67.CrossRef
19.
go back to reference Pfeiffenberger J, Beinhardt S, Gotthardt DN, et al. Pregnancy in Wilson disease: management and outcome. Hepatology. 2018;67:1261–9.CrossRef Pfeiffenberger J, Beinhardt S, Gotthardt DN, et al. Pregnancy in Wilson disease: management and outcome. Hepatology. 2018;67:1261–9.CrossRef
20.
go back to reference Ferenci P. Pathophysiology and clinical features of Wilson disease. Metab Brain Dis. 2004;19:229–39.CrossRef Ferenci P. Pathophysiology and clinical features of Wilson disease. Metab Brain Dis. 2004;19:229–39.CrossRef
Metadata
Title
The study of Wilson disease in pregnancy management
Authors
Xu-En Yu
Min Pan
Yong-Zhu Han
Ren-Min Yang
Juan Wang
Shan Gao
Publication date
01-12-2019
Publisher
BioMed Central
Published in
BMC Pregnancy and Childbirth / Issue 1/2019
Electronic ISSN: 1471-2393
DOI
https://doi.org/10.1186/s12884-019-2641-8

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