Pregnancy with Wilson’s disease
DOI:
https://doi.org/10.18203/2320-1770.ijrcog20252890Keywords:
Copper, Miscarriages, Copper chelating agent, Zinc sulphate, Wilsons diseaseAbstract
Wilson’s disease is a rare, autosomal recessive disorder characterized by impaired liver metabolism of copper, leading to decreased biliary excretion and incorporation of ceruloplasmin levels mainly in the liver and brain. Untreated Wilson’s disease has been shown to cause subfertility and miscarriages. Pregnancy management in women with Wilson’s disease remains an important clinical problem. Early recognition and effective management can help prevent the disease progression and hence give eventful progress and perinatal outcomes. We hereby report the case of a 29-year-old pregnant multigravida with known case of Wilson’s disease since childhood, and on treatment with copper chelating agents and zinc sulphate. She was followed up from early pregnancy till delivery at our hospital. She was monitored in combined obstetric, medicine and neurology clinic. Her pregnancy and postpartum course remained uneventful. She had a spontaneous vaginal delivery at 38+5 weeks period of gestation and gave birth to healthy female baby of 2.66 kg in weight. The infant was genetically screened and was found to be carrier free. It is relatively safe for women with Wilsons disease to become pregnant. Patients with Wilson’s disease receiving regular treatment who remain asymptomatic are usually able to conceive and achieve successful outcomes. However, these pregnancies should be considered as high risk and merit regular surveillance.
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Malik A, Khawaja A, Sheikh L. Wilson's disease in pregnancy: case series and review of literature. BMC Res Notes. 2013;6:421. DOI: https://doi.org/10.1186/1756-0500-6-421
Pfeiffer RF. Wilson's Disease. Semin Neurol. 2007;27(2):123-32. DOI: https://doi.org/10.1055/s-2007-971173
Gibbs K, Walshe JM. A study of the caeruloplasmin concentrations found in 75 patients with Wilson's disease, their kinships and various control groups. Q J Med. 1979;48(191):447-63.
Frydman M. Genetic aspects of Wilson's disease. J Gastroenterol Hepatol. 1990;5(4):483-90. DOI: https://doi.org/10.1111/j.1440-1746.1990.tb01427.x
Sinha S, Taly AB, Prashanth LK, Arunodaya GR, Swamy HS. Successful pregnancies and abortions in symptomatic and asymptomatic Wilson's disease. J Neurol Sci. 2004;217(1):37-40. DOI: https://doi.org/10.1016/j.jns.2003.08.007
Tarnacka B, Rodo M, Cichy S, Członkowska A. Procreation ability in Wilson's disease. Acta Neurol Scand. 2000;101(6):395-8. DOI: https://doi.org/10.1034/j.1600-0404.2000.90140a.x
Cumings JN. The effects of B.A.L. in hepatolenticular degeneration. Brain. 1951;74(1):10-22. DOI: https://doi.org/10.1093/brain/74.1.10
Hung IH, Suzuki M, Yamaguchi Y, Yuan DS, Klausner RD, Gitlin JD. Biochemical characterization of the Wilson disease protein and functional expression in the yeast Saccharomyces cerevisiae. J Biol Chem. 1997;272(34):21461-6. DOI: https://doi.org/10.1074/jbc.272.34.21461
Scheinberg IH, Sternlieb I. Wilson's disease. Annu Rev Med. 1965;16:119-34. DOI: https://doi.org/10.1146/annurev.me.16.020165.001003
Brewer GJ. Recognition, diagnosis, and management of Wilson's disease. Proc Soc Exp Biol Med. 2000;223(1):39-46. DOI: https://doi.org/10.1046/j.1525-1373.2000.22305.x
Mustafa MS, Shamina AH. Five successful deliveries following 9 consecutive spontaneous abortions in a patient with Wilson disease. Aust N Z J Obstet Gynaecol. 1998;38(3):312-4. DOI: https://doi.org/10.1111/j.1479-828X.1998.tb03073.x
Morimoto I, Ninomiya H, Komatsu K, Satho M. Pregnancy and penicillamine treatment in a patient with Wilson's disease. Japan J Med. 1986;25(1):59-62. DOI: https://doi.org/10.2169/internalmedicine1962.25.59
Scheinberg IH, Sternlieb I. Pregnancy in penicillamine-treated patients with Wilson's disease. N Engl J Med. 1975;293(25):1300-2. DOI: https://doi.org/10.1056/NEJM197512182932507
Oster G, Salgo MP. The copper intrauterine device and its mode of action. N Engl J Med. 1975;293(9):432-8. DOI: https://doi.org/10.1056/NEJM197508282930906
Pfeiffenberger J. Pregnancy in Wilson's disease: Management and outcome. Hepatology. 2018;67(4):1261-9. DOI: https://doi.org/10.1002/hep.29490
Haggstrom GD, Hirschowitz BI, Flint A. Long-term penicillamine therapy for Wilson's disease. South Med J. 1980;73(4):530-1. DOI: https://doi.org/10.1097/00007611-198004000-00040
Nazer H, Ede RJ, Mowat AP, Williams R. Wilson's disease in childhood. Variability of clinical presentation. Clin Pediatr (Phila). 1983;22(11):755-7. DOI: https://doi.org/10.1177/000992288302201104
Sternlieb I, Scheinberg IH. Penicillamine therapy for hepatolenticular degeneration. JAMA. 1964;189:748-54. DOI: https://doi.org/10.1001/jama.1964.03070100042008
Walshe JM. The management of pregnancy in Wilson's disease treated with trientine. Q J Med. 1986;58(225):81-7.
Dupont P, Irion O, Béguin F. Pregnancy in a patient with treated Wilson's disease: a case report. Am J Obstet Gynecol. 1990;163(5):1527-8. DOI: https://doi.org/10.1016/0002-9378(90)90619-I
Walshe JM. Wilson's disease. The presenting symptoms. Arch Dis Child. 1962;37(193):253-6. DOI: https://doi.org/10.1136/adc.37.193.253
Hoogenraad TU. Paradigm shift in treatment of Wilson's disease: zinc therapy now treatment of choice. Brain Dev. 2006;28(3):141-6. DOI: https://doi.org/10.1016/j.braindev.2005.08.008
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