Saito Kana, Onishi Eiko, Itagaki Jun, Toda Noriko, Haitani Azusa, Yamauchi Masanori
Department of Anesthesiology, Tohoku University Hospital, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, 980-8574, Japan.
Department of Anesthesiology, Tohoku Rosai Hospital, 4-3-21 Dainohara, Aoba-ku, Sendai, Miyagi, 981-8563, Japan.
JA Clin Rep. 2019 Nov 13;5(1):75. doi: 10.1186/s40981-019-0294-2.
Wilson's disease is a rare autosomal recessive disorder affecting copper metabolism, which presents liver and brain dysfunction caused by abnormal copper accumulation. We report a patient who showed exacerbation of liver failure during pregnancy.
A 24-year-old woman with Wilson's disease was scheduled for emergency cesarean delivery at 30 weeks of gestation. The patient exhibited severe coagulopathy and prominent body weight gain (+ 30 kg) caused by systemic edema and ascites. We decided to perform emergency cesarean delivery under general anesthesia. We used platelet concentrates, cryoprecipitate, and fibrinogen concentrate. Intraoperative hemorrhage was well controlled. On the 15th postpartum day, weight was reduced by 20 kg and liver function had improved. She and her baby were discharged without complications.
The appropriate continued treatment of Wilson's disease and supplementation of coagulation factors and/or platelets when indicated greatly increase the likelihood of a successful pregnancy, even in patients with liver failure exacerbation.
威尔逊病是一种罕见的常染色体隐性疾病，影响铜代谢，可导致铜异常蓄积引起肝脏和脑功能障碍。我们报告一例在孕期出现肝衰竭加重的患者。
一名患有威尔逊病的24岁女性在妊娠30周时计划行急诊剖宫产。患者表现出严重的凝血功能障碍，因全身水肿和腹水导致体重显著增加（增加30千克）。我们决定在全身麻醉下进行急诊剖宫产。我们使用了浓缩血小板、冷沉淀和纤维蛋白原浓缩物。术中出血得到了良好控制。产后第15天，体重减轻了20千克，肝功能有所改善。她和她的婴儿顺利出院，无并发症。
即使对于肝衰竭加重的患者，适当持续治疗威尔逊病并在必要时补充凝血因子和/或血小板，可大大增加成功妊娠的可能性。
