The clinical course of a pregnancy complicated by Wilson disease.
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This case report describes a successful pregnancy in a 23-year-old patient with Wilson's disease (hapatolenticicular degeneration). This patient had onset of clinical singns of Wilson's disease at the age of 20, then showed the triad of extrapyramidal tract signs, liver chirrosis, and Kayser-Fleischer ring, complicated secondary amenorrhea at the age of 22. Endocrinological studies revealed a polycystic ovary syndrome (PCOS) like pattern. Treatment with D-penicillamine improved her clinical symptoms, and she become pregnant without use of ovulation-inducing agents.<BR>From 10w to 24w of gestation, D-penicillamine was discontinued, then platelet count and serum cholinesterase activity were then reduced. D-penicillamine therapy was then reinstituted with 500mg/day p. o., and urinary excretion of copper, platelet count and serum cholinasterase were improved.<BR>At 26w and 36w of gestation, esophageal endoscopic studies revealed no presence of active esophageal varices. At 38w 6days of gestation, she was vaginally delivered of a normal female infant with an Apgar score of 9 and weighing 3, 015g.<BR>Authers emhasize the following concerning the management of Wilson's disease in pregnancy : <BR>1) Good control of Willson's disease with the administration of D-penicillamine therapy before pregnancy.<BR>2) Continuation of D-penicillamine therapy during pregnancy.<BR>3) Reduction of the dose of D-penicillamine during pregnancy.<BR>4) Elaborate investigation of esophageal varices.
- 近畿産科婦人科学会の論文
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