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替代疗法对血友病携带者妊娠结局的影响:沙特阿拉伯的一项历史性队列研究

Impact of Replacement Therapy on Pregnancy Outcomes in Hemophilia Carriers: A Historical Cohort Study in Saudi Arabia.

作者信息

Bakhsh Ebtisam

机构信息

Internal Medicine Department, College of Medicine, Princess Nourah bint Abdulrahman University, Riyadh 11564, Saudi Arabia.

出版信息

Life (Basel). 2024 May 11;14(5):623. doi: 10.3390/life14050623.

Abstract

This retrospective cohort study evaluates the safety and efficacy of replacement therapy with regard to pregnancy outcomes in hemophilia carriers. Hemophilia carriers face elevated bleeding risks during pregnancy, necessitating meticulous management, including replacement therapy with clotting factors. This research examines the records of 64 pregnant hemophilia carriers at King Fahad Medical City, Riyadh, from January 2010 to December 2023, analyzing their demographic details, hemophilia type and severity, replacement therapy specifics, and pregnancy outcomes. The study found that 62.5% of the participants had hemophilia A, with 43.8% categorized as severe. Most subjects (87.5%) received recombinant factor VIII at a median dosage of 30 IU/kg weekly. Adverse pregnancy outcomes included gestational hypertension (15.6%), preterm labor (18.8%), and postpartum hemorrhage (12.5%). The cesarean section rate was 28.1%. Neonatal outcomes were generally favorable, with median birth weights at 3100 g and mean Apgar scores of 8.2 and 9.1 at 1 and 5 min, respectively. Logistic regression analysis revealed no significant association between adverse events and therapy type or dosage, though a trend towards significance was noted with once-weekly administration ( = 0.082). The study concludes that replacement therapy is a viable method for managing hemophilia in pregnant carriers, leading to generally favorable maternal and neonatal outcomes. However, it underscores the importance of individualized treatment plans and close monitoring to effectively manage the risks associated with hemophilia during pregnancy.

摘要

这项回顾性队列研究评估了替代疗法在血友病携带者妊娠结局方面的安全性和有效性。血友病携带者在怀孕期间面临更高的出血风险,因此需要精心管理,包括使用凝血因子进行替代疗法。本研究调查了利雅得法赫德国王医疗城2010年1月至2023年12月期间64名怀孕血友病携带者的记录,分析了她们的人口统计学细节、血友病类型和严重程度、替代疗法的具体情况以及妊娠结局。研究发现,62.5%的参与者患有A型血友病,其中43.8%为重度。大多数受试者(87.5%)接受重组因子VIII治疗,每周中位剂量为30 IU/kg。不良妊娠结局包括妊娠期高血压(15.6%)、早产(18.8%)和产后出血(12.5%)。剖宫产率为28.1%。新生儿结局总体良好,中位出生体重为3100 g,1分钟和5分钟时的平均阿氏评分分别为8.2和9.1。逻辑回归分析显示,不良事件与治疗类型或剂量之间无显著关联,不过每周一次给药有显著趋势(P = 0.082)。该研究得出结论,替代疗法是管理怀孕携带者血友病的一种可行方法,可带来总体良好的母婴结局。然而,它强调了个性化治疗方案和密切监测对于有效管理怀孕期间与血友病相关风险的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8db3/11122275/6246dbefed7f/life-14-00623-g001.jpg

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