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与妊娠晚期出血相关的先天性低纤维蛋白原血症

Constitutional hypofibrinogenemia associated with third trimester hemorrhage.

作者信息

McRoyan D K, McRoyan C J, Kitay D Z, Liu P I

出版信息

Ann Clin Lab Sci. 1986 Jan-Feb;16(1):52-7.

PMID:3947029
Abstract

This case of a 31-year-old white woman presenting at 32 weeks gestation with vaginal bleeding supports a possible association between constitutional hypofibrinogenemia and third trimester hemorrhage. Clot based fibrinogen assays were persistently low for the third trimester (patient: 102 to 155 mg per dl; normal range: 400 to 650 mg per dl) and at follow-up, ten months post-partum (patient: 90 mg per dl; normal range 180 to 436 mg per dl); an immunologic fibrinogen level was comparable. The patient gave an unremarkable bleeding history. Of 11 previously reported pregnancies in five hypofibrinogenemic patients, six terminated in placental abruption during the third trimester, two others were complicated by significant post-partum hemorrhage, and one by spontaneous abortion. This case report emphasizes that low functional levels of fibrinogen are potentially disruptive to the integrity of the uteroplacental interface. The pregnant state unmasks and amplifies an otherwise silent to mild hemostatic disorder.

摘要

该病例为一名31岁白人女性,妊娠32周时出现阴道出血,提示先天性低纤维蛋白原血症与孕晚期出血之间可能存在关联。基于凝血块的纤维蛋白原检测在孕晚期持续偏低(患者:每分升102至155毫克;正常范围:每分升400至650毫克),产后10个月随访时仍低(患者:每分升90毫克;正常范围每分升180至436毫克);免疫纤维蛋白原水平相当。患者既往出血史无异常。在先前报道的5例低纤维蛋白原血症患者的11次妊娠中,6例在孕晚期因胎盘早剥而终止妊娠,另外2例并发严重产后出血,1例自然流产。本病例报告强调,纤维蛋白原功能水平低可能会破坏子宫胎盘界面的完整性。妊娠状态会暴露并放大原本无症状或症状轻微的止血障碍。

相似文献

1
Constitutional hypofibrinogenemia associated with third trimester hemorrhage.与妊娠晚期出血相关的先天性低纤维蛋白原血症
Ann Clin Lab Sci. 1986 Jan-Feb;16(1):52-7.
2
Retrochorionic hematoma in congenital afibrinogenemia: resolution with fibrinogen concentrate infusions.先天性纤维蛋白原血症中的绒毛膜后血肿:通过输注纤维蛋白原浓缩物得以消退。
Am J Hematol. 2007 Apr;82(4):317-20. doi: 10.1002/ajh.20802.
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Spontaneous resolution of hypofibrinogenemia in a triplet gestation associated with second trimester in utero death of two fetuses.
Am J Perinatol. 1993 Nov;10(6):448-9. doi: 10.1055/s-2007-994628.
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The outcome of pregnancies complicated by bleeding during the second trimester.妊娠中期并发出血的妊娠结局。
Surg Gynecol Obstet. 1991 Nov;173(5):371-4.
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Treatment of patients with dysfibrinogenemia and a history of abortions during pregnancy.对患有异常纤维蛋白原血症且有孕期流产史的患者进行治疗。
Blood Coagul Fibrinolysis. 2009 Jul;20(5):366-70. doi: 10.1097/MBC.0b013e32832aec2b.
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Pregnancy-related thrombosis in a woman with congenital afibrinogenemia: a report of two successful pregnancies.一名先天性无纤维蛋白原血症女性的妊娠相关血栓形成:两例成功妊娠的报告
Am J Hematol. 2004 Jul;76(3):267-70. doi: 10.1002/ajh.20110.
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Congenital hypofibrinogenemia and recurrent placental abruption.先天性低纤维蛋白原血症与复发性胎盘早剥。
Obstet Gynecol. 1983 Apr;61(4):519-23.
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Coagulation-fibrinolysis is more enhanced in twin than in singleton pregnancies.双胎妊娠的凝血-纤溶系统比单胎妊娠更活跃。
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Dysfibrinogenemia associated with thrombosis and third-trimester fetal loss. A case report.伴有血栓形成和孕晚期胎儿丢失的异常纤维蛋白原血症。病例报告。
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Urethral varices as an unusual cause of third-trimester bleeding. A case report.尿道静脉曲张作为孕晚期出血的罕见原因:一例报告
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引用本文的文献

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Hypofibrinogenemia and miscarriage: report of a first successful pregnancy under fibrinogen substitution and short review of the literature.低纤维蛋白原血症与流产:首例在纤维蛋白原替代治疗下成功妊娠的报告及文献综述
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