表现为严重蛋白C缺乏和围产期血栓形成的杂合子蛋白C缺乏症：蛋白C浓缩物治疗成功。
Heterozygous protein C deficiency presenting as severe protein C deficiency and peripartum thrombosis: successful treatment with protein C concentrate.
作者信息
Goodwin T M, Gazit G, Gordon E M
机构信息
Department of Obstetrics and Gynecology, Women's and Children's Hospital, University of Southern California, Los Angeles, USA.
出版信息
Obstet Gynecol. 1995 Oct;86(4 Pt 2):662-4. doi: 10.1016/0029-7844(95)00039-t.
BACKGROUND
Complications of pregnancy, including thrombosis and increased fetal loss, have been described in women with heterozygous protein C deficiency.
CASE
We report an unusual case of umbilical cord thrombosis, acute renal failure, and cutaneous venous thrombosis associated with severe protein C deficiency in the heterozygous infant of a normal mother. Repeated infusions of protein C concentrate made successful heparinization of the infant possible, with the subsequent resolution of cutaneous thrombosis and improvement of renal status.
CONCLUSION
Heterozygous protein C deficiency in the fetus may result in perinatal thrombosis, which can be treated with viral-inactivated protein C concentrate. Heterozygous protein C deficiency should be considered in cases of fetal compromise or death associated with umbilical vein thrombosis.
背景
杂合子蛋白C缺乏症女性患者存在包括血栓形成和胎儿丢失增加在内的妊娠并发症。
病例
我们报告了一例异常病例，一名正常母亲的杂合子婴儿患有严重蛋白C缺乏症，伴有脐带血栓形成、急性肾衰竭和皮肤静脉血栓形成。反复输注蛋白C浓缩物使婴儿成功肝素化成为可能，随后皮肤血栓溶解，肾功能改善。
结论
胎儿杂合子蛋白C缺乏可能导致围产期血栓形成，可用病毒灭活的蛋白C浓缩物治疗。对于与脐静脉血栓形成相关的胎儿窘迫或死亡病例，应考虑杂合子蛋白C缺乏症。
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