Management of Pregnancy, Delivery, and Postpartum in Italian Carriers and Women With Haemophilia A and B

IF 3 2区 医学 Q2 HEMATOLOGY
Haemophilia Pub Date : 2025-07-29 DOI:10.1111/hae.70097
Silvia Linari, Berardino Pollio, Antonietta Ferretti, Mariasanta Napolitano, Elvira Grandone, Simona Siboni, Anna Brigida Aru, Elisa Bertoni, Chiara Biasoli, Isabella Cantori, Patrizia Di Gregorio, Giuseppe Lassandro, Gianluca Sottilotta, Alessandra Strangio, Maria Rosaria Villa, Giancarlo Castaman, Angiola Rocino, Italian Association of Hemophilia Centers MEC donna Working Group
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引用次数: 0

Abstract

Introduction

Managing pregnancy in carriers/women with haemophilia involves four key objectives: preconception and prenatal care, minimizing maternal bleeding, choosing the delivery mode to reduce foetal/neonatal bleeding, and postpartum care. The optimal factor VIII/IX level at delivery and the best delivery method to mitigate maternal and foetal complications are still debated.

Aim

To share real-world clinical management in Italian haemophilia treatment centres.

Methods

In March-April 2023, members of the Italian Association of Hemophilia Centers “AICE” were invited to an online survey to collect information on the management of pregnancy, delivery, and postpartum in carriers/women with haemophilia.

Results

Thirty-three centres responded. Plasma FVIII/FIX levels are systematically evaluated in the third trimester, considering a threshold above 50 U/dL essential for safe delivery and neuraxial analgesia. Overall, the haematologic counselling on delivery mode generally aligns with obstetric indications in 73% of centres. In women who will give birth to a known affected male foetus, 55% rely on obstetric indication, while 24% suggest spontaneous delivery and 21% planned caesarean section. Excluding those which follow obstetric indication, natural delivery and planned caesarean section are recommended respectively by 40% and 60% when prenatal diagnosis is available or by 20% and 80% when not available. All centres agree to avoid instrumental delivery in affected male foetus or when prenatal diagnosis has not been made.

Conclusion

Multidisciplinary pregnancy management is recommended. Delivery mode choice is primarily driven by ensuring the newborn's maximum protection. However, the use of caesarean section should also be weighted according to the mother's risk profile.

Abstract Image

意大利A型和B型血友病携带者和妇女的妊娠、分娩和产后管理
导论:血友病携带者/妇女的妊娠管理涉及四个关键目标:孕前和产前护理,尽量减少产妇出血,选择分娩方式以减少胎儿/新生儿出血,以及产后护理。分娩时的最佳因子VIII/IX水平和减轻母婴并发症的最佳分娩方法仍存在争议。目的:分享现实世界的临床管理在意大利血友病治疗中心。方法:在2023年3月至4月,邀请意大利血友病中心协会(AICE)的成员进行在线调查,收集血友病携带者/妇女的妊娠、分娩和产后管理信息。结果:33个中心回应。在妊娠晚期系统评估血浆FVIII/FIX水平,考虑到50 U/dL以上的阈值对于安全分娩和神经轴镇痛至关重要。总体而言,在73%的中心,关于分娩方式的血液学咨询通常与产科指征一致。在将生下已知受影响男性胎儿的妇女中,55%依赖产科指征,24%建议自然分娩,21%建议计划剖宫产。不包括那些遵循产科指征的人,在有产前诊断的情况下,建议自然分娩和计划剖腹产的比例分别为40%和60%,在没有产前诊断的情况下,建议自然分娩和计划剖腹产的比例分别为20%和80%。所有中心都同意避免对受影响的男性胎儿或未进行产前诊断时进行器械分娩。结论:推荐多学科妊娠管理。分娩方式的选择主要是为了确保新生儿得到最大程度的保护。然而,剖腹产的使用也应根据母亲的风险概况进行权衡。
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来源期刊
Haemophilia
Haemophilia 医学-血液学
CiteScore
6.50
自引率
28.20%
发文量
226
审稿时长
3-6 weeks
期刊介绍: Haemophilia is an international journal dedicated to the exchange of information regarding the comprehensive care of haemophilia. The Journal contains review articles, original scientific papers and case reports related to haemophilia care, with frequent supplements. Subjects covered include: clotting factor deficiencies, both inherited and acquired: haemophilia A, B, von Willebrand''s disease, deficiencies of factor V, VII, X and XI replacement therapy for clotting factor deficiencies component therapy in the developing world transfusion transmitted disease haemophilia care and paediatrics, orthopaedics, gynaecology and obstetrics nursing laboratory diagnosis carrier detection psycho-social concerns economic issues audit inherited platelet disorders.
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