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首页> 外文期刊>The journal of obstetrics and gynaecology research >Case of maternal and fetal deaths due to severe congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) during pregnancy
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Case of maternal and fetal deaths due to severe congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) during pregnancy

机译：怀孕期间因严重的先天性血栓性血小板减少性紫癜（Upshaw-Schulman综合征）导致的母婴死亡案例

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Upshaw-Schulman syndrome (USS) involves a congenital deficiency of von Willebrand factor-cleaving metalloprotease (ADAMTS13) activity due to gene mutations. Female patients develop overt thrombotic thrombocytopenic purpura (TTP) caused by a decline of ADAMTS13 activity in pregnancy.A 23-year-old nulliparous Japanese woman died due to severe, rapid progression of TTP with intrauterine fetal death at 20 weeks of gestation after its onset, even though she underwent intensive treatment which included plasma exchange. She had a history of idiopathic thrombocytopenic purpura at the age of 3 years. The patient's ADAMTS13 activity was of very low level. It should be borne in mind that there is the possibility of rapidly progressive fulminant USS during pregnancy.
机译：Upshaw-Schulman综合征（USS）涉及由于基因突变导致的先天性von Willebrand因子切割金属蛋白酶（ADAMTS13）活性不足。女性患者会因妊娠ADAMTS13活性下降而出现明显的血栓性血小板减少性紫癜（TTP）.23岁的日本未产妇死于TTP的严重，快速进展并在其发病20周后死于子宫内胎儿。 ，即使她接受了包括血浆置换在内的强化治疗。她有3岁的特发性血小板减少性紫癜病史。患者的ADAMTS13活性很低。应该记住的是，怀孕期间可能会迅速进行暴发性USS。

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