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[血友病携带者:一家法国大学医院的经验]

[Carriers of haemophilia: Experience of a French university hospital].

作者信息

Sauguet P, Aguilar-Martinez P, Boulot P, Escudié J-B, Schved J-F, Biron-Andréani C

机构信息

Département d'hématologie biologique et centre régional de traitement de l'hémophilie, pôle biologie-pathologie, hôpital Saint-Eloi, CHRU Montpellier, 80, avenue Augustin-Fliche, 34295 Montpellier cedex 05, France.

Département de gynécologie-obstétrique, pôle naissance et pathologies de la femme, hôpital Arnaud-de-Villeneuve, CHRU Montpellier, 37, avenue du Doyen-Gaston-Giraud, 34295 Montpellier cedex 05, France.

出版信息

J Gynecol Obstet Biol Reprod (Paris). 2015 Jun;44(6):565-76. doi: 10.1016/j.jgyn.2014.08.009. Epub 2014 Sep 26.

Abstract

OBJECTIVES

To report the management of carriers of haemophilia in a French university hospital and assess different issues of these patients.

PATIENTS AND METHODS

Retrospective study of the carriers of haemophilia who consulted at the university hospital of Montpellier, France, between 1995 and 2011. Information were obtained from medical records and from a questionnaire sent to carriers. We recorded data about biological characteristics, bleeding tendency and management of pregnancies.

RESULTS

Sixty-four carriers of haemophilia A or B were included. Their median FVIII or FIX level was 52 % (range, 15-137 %). Menstrual bleeding lasted more than 7 days in 31 % of carriers. A total of 142 pregnancies started in 54 carriers, and 101 resulted in live births with 26 boys with haemophilia. Sixty-two prenatal diagnoses carried out, 15 have terminated their pregnancy because of a hemophiliac male fetus. Seventy-six percent of deliveries were vaginal delivery and 49 % took place in a level-3 maternity. There were 10.8 % and 8.5 % primary and secondary post-partum hemorrhage, respectively.

CONCLUSION

The risk of bleeding among carriers of haemophilia is associated with their antihemophilic factor level. To improve the management of carriers, a multidisciplinary and standardized medical record, with a specific questionnaire to evaluate bleedings, could be considered. A regional register that lists all carriers, regardless of their antihemophilic factor level, would also be useful.

摘要

目的

报告法国一家大学医院中血友病携带者的管理情况,并评估这些患者的不同问题。

患者与方法

对1995年至2011年间在法国蒙彼利埃大学医院就诊的血友病携带者进行回顾性研究。信息来自病历以及发送给携带者的一份问卷。我们记录了有关生物学特征、出血倾向和妊娠管理的数据。

结果

纳入了64例甲型或乙型血友病携带者。他们的FVIII或FIX水平中位数为52%(范围为15% - 137%)。31%的携带者月经出血持续超过7天。54名携带者共开始了142次妊娠,其中101次分娩出活产婴儿,有26名患血友病的男孩。进行了62次产前诊断,15例因男性血友病胎儿而终止妊娠。76%的分娩为阴道分娩,49%在三级产科进行。分别有10.8%和8.5%的产妇发生原发性和继发性产后出血。

结论

血友病携带者的出血风险与其抗血友病因子水平有关。为改善对携带者的管理,可以考虑建立一份多学科的标准化病历,并使用一份特定问卷来评估出血情况。建立一个列出所有携带者(无论其抗血友病因子水平如何)的区域登记册也会很有用。

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