因子 XIII A 亚单位重组体在因子 XIII 缺乏和复发性流产患者中的应用。
Recombinant factor XIII A-subunit in a patient with factor XIII deficiency and recurrent pregnancy loss.
机构信息
Department of Hematology, Sultan Qaboos University Hospital, Muscat, Oman.
Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico and Luigi Villa Foundation, Milan, Italy.
出版信息
J Thromb Haemost. 2018 Jun;16(6):1052-1054. doi: 10.1111/jth.14126. Epub 2018 May 13.
UNLABELLED
Essentials Inherited factor XIII deficiency is a very rare bleeding disorder. We used recombinant factor XIII-A in a pregnant patient with factor XIII-A subunit deficiency. The patient had a successful pregnancy outcome with no pregnancy related complications. The dose of recombinant factor XIII-A was minimized by using frequent trough level monitoring.
SUMMARY
Inherited factor XIII deficiency is a very rare bleeding disorder, and is one of the causes of recurrent pregnancy loss. The use of plasma-derived FXIII to improve pregnancy outcomes has been reported. We report a 26-year-old woman with FXIII A-subunit (FXIII-A) deficiency who was treated with recombinant FXIII-A and had a successful pregnancy outcome with no pregnancy-related complications. Our case illustrates that the dose of recombinant FXIII-A can be minimized and adjusted on the basis of frequent trough level monitoring.
未注明
遗传性因子 XIII 缺乏症是一种非常罕见的出血性疾病。我们在一名因子 XIII-A 亚单位缺陷的孕妇中使用了重组因子 XIII-A。该患者成功妊娠，无妊娠相关并发症。通过频繁的谷底水平监测，使重组因子 XIII-A 的剂量最小化。
摘要
遗传性因子 XIII 缺乏症是一种非常罕见的出血性疾病，也是复发性流产的原因之一。已报道使用血浆衍生的 FXIII 来改善妊娠结局。我们报告了一名 26 岁的女性，患有 FXIII A 亚单位（FXIII-A）缺乏症，接受了重组 FXIII-A 治疗，成功妊娠，无妊娠相关并发症。我们的病例表明，可以根据频繁的谷底水平监测来最小化和调整重组 FXIII-A 的剂量。
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