Anaesthetic Management in a Case of Wilson's Disease in Pregnancy

B. Mohanty
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引用次数: 2

Abstract

Wilson’s disease (WD) is an inherited autosomal recessive disease results due to deficiency of ceruloplasmin leading to copper deposition mainly liver, brain and cornea. This accumulation can cause liver cirrhosis, ataxia and kayser Fleischer ring (KF) in cornea. I present a case of 21-year-old primigravida diagnosed WD 3 years back on treatment with Zinc was referred to our hospital for elective caesarean section (CS). She had thrombocytopenia, extrahepatic portal vein obstruction with hypersplenism and mild ascites. She has undergone CS under general anaesthesia (GA) with successful outcome. The anaesthetic management in presence of hepatic dysfunction and thrombocytopenia was challenging and there are only few case reports on WD patients undergoing CS under GA.
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妊娠期威尔逊氏病1例的麻醉处理
威尔逊氏病(WD)是一种遗传性常染色体隐性遗传病,由于铜蓝蛋白缺乏导致铜沉积,主要发生在肝脏、脑和角膜。这种积累可引起肝硬化、共济失调和角膜的凯泽-弗莱舍环(KF)。我报告一个21岁的初产妇诊断为WD 3年前治疗锌被转介到我院择期剖宫产(CS)。她有血小板减少,肝外门静脉阻塞伴脾功能亢进和轻度腹水。她在全身麻醉(GA)下接受了CS,结果成功。存在肝功能障碍和血小板减少的麻醉管理具有挑战性,只有少数WD患者在GA下接受CS的病例报道。
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