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PREGNANCY IN A WOMAN WITH WILSON’S DISEASE TREATED WITH ZINC: A CASE REPORT

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초록

Wilson’s disease is a rare hereditary disorder of copper metabolism affecting 30 per million populations. Copper get deposited in liver, brain and kidney due to decreased hepato-cellular excretion and leading to diverse clinical manifestations. Patient may remain apparently asymptomatic or may present with fulminant liver disease or neuropsychiatric illness. Reproductive outcome in untreated Wilson’s disease is poor. Women remaining untreated either suffer from infertility or experience recurrent pregnancy losses. Invention of copper chelating agents and their usage in these women resulted in successful pregnancy outcome. Penicillamine or zinc salts can be used as copper chelator in pregnancy with Wilson’s disease with equal effectiveness in respect to pregnancy outcome. We report a case of previously undiagnosed Wilson’s disease with three consecutive pregnancy losses who achieved a live birth after successfully treated with zinc salts. The several points regarding antenatal care, drug therapy and optimum time and mode of delivery for the woman with Wilson’s disease are discussed here.

Abstract

Wilson’s disease is a rare hereditary disorder of copper metabolism affecting 30 per million populations. Copper get deposited in liver, brain and kidney due to decreased hepato-cellular excretion and leading to diverse clinical manifestations. Patient may remain apparently asymptomatic or may present with fulminant liver disease or neuropsychiatric illness. Reproductive outcome in untreated Wilson’s disease is poor. Women remaining untreated either suffer from infertility or experience recurrent pregnancy losses. Invention of copper chelating agents and their usage in these women resulted in successful pregnancy outcome. Penicillamine or zinc salts can be used as copper chelator in pregnancy with Wilson’s disease with equal effectiveness in respect to pregnancy outcome. We report a case of previously undiagnosed Wilson’s disease with three consecutive pregnancy losses who achieved a live birth after successfully treated with zinc salts. The several points regarding antenatal care, drug therapy and optimum time and mode of delivery for the woman with Wilson’s disease are discussed here.

참고문헌 (5)

  1. Roberts EA, "Diagnosis and treatment of Wilson disease: an update. Hepatology", Hepatology, 47, pp.2089~2111 (2008)
  2. Sinha S, "Successful pregnancies and abortions in symptomatic and asymptomatic Wilson’s disease", J Neurol Sci, 217, pp.37~40 (2004)
  3. Brewer GJ, "Treatment of Wilson’s disease with zinc. XVII: treatment during pregnancy", Hepatology, 31, pp.364~370 (2000)
  4. Scheinberg IH, "Pregnancy in penicillamine-treated patients with Wilson’s disease", N Engl J Med, 293, pp.1300~1302 (1975)
  5. Kroll CA, "Retrospective determination of ceruloplasmin in newborn screening blood spots of patients with Wilson disease", Mol Genet Metab, 89, pp.134~138 (2006)

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