妊娠伴Wilson病:一个病例系列。

Mymensingh medical journal : MMJ Pub Date : 2025-01-01
M Saha, S Hayee, S Sayeeda, T Parveen, N Akhtar
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引用次数: 0

摘要

威尔逊氏病是一种常染色体隐性遗传病,由于铜蓝蛋白缺乏而影响铜的运输,导致铜沉积主要在肝脏、大脑和角膜。它引起肝脏和/或神经精神症状。这种铜沉积导致肝硬化、脑病和肝功能衰竭。它还会导致月经周期紊乱和生育能力低下。在怀孕期间,发生先兆子痫、胎儿生长受限和胎儿神经损伤的风险增加。妊娠伴失代偿性肝硬化会出现更多并发症,如食管静脉曲张出血、肝功能衰竭、脑病和脾动脉破裂。一个成功的去铜剂可能导致恢复排卵周期和使怀孕。我们报告了4例已知的威尔逊病伴妊娠的成功妊娠结果,这些病例来自Bangabandhu Sheikh Mujib医科大学(BSMMU)的胎母医学系,并讨论了患有这种疾病的孕妇的管理。
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Pregnancy with Wilson's Disease: A Case Series.

Wilson's disease is an autosomal recessive disorder that affects copper transport due to deficiency of ceruloplasmin and causes deposition of copper mainly in the liver, brain and cornea. It causes hepatic and/or neuropsychiatric manifestations. This copper deposition causes cirrhosis of the liver, encephalopathy and liver failure. It also causes disorders of the menstrual cycle and subfertility. During pregnancy, there is an increased risk of the development of preeclampsia, fetal growth restriction, and neurologic damages to the fetus. Pregnancy with decompensated liver cirrhosis developed more complications like bleeding from esophageal varices, liver failure, encephalopathy, and rupture of the splenic artery. A successful decopperizing agent may lead to restoration of the ovulatory cycle and enable pregnancy. We are reporting four cases of successful pregnancy outcomes of known case of Wilson's disease with pregnancy which was taken from Fetomaternal Medicine Department of Bangabandhu Sheikh Mujib Medical University (BSMMU), and discussing the management of pregnant women with this disorder.

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