Sites of initial bleeding episodes, mode of delivery and age of diagnosis in babies with haemophilia diagnosed before the age of 2 years: a report from The Centers for Disease Control and Prevention's (CDC) Universal Data Collection (UDC) project
Sites of initial bleeding episodes, mode of delivery and age of diagnosis in babies with haemophilia diagnosed before the age of 2 years: a report from The Centers for Disease Control and Prevention's (CDC) Universal Data Collection (UDC) project
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DOI：
10.1111/j.1365-2516.2009.02074.x
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发表时间：
2009-11-01
期刊：
影响因子：
3.9
通讯作者：
Evatt, B.
中科院分区：
文献类型：
--
作者：
Kulkarni, R.;Soucie, J. M.;Evatt, B.
缺乏血友病新生儿和幼儿详细的自然史和结果数据阻碍了对该疾病的最佳治疗。我们报告了对参加美国疾病控制与预防中心 (CDC) 通用数据收集 (UDC) 监测项目的 580 名 0-2 岁血友病新生儿和幼儿收集的前瞻性数据的分析。这项研究的重点是一群两岁前确诊的血友病婴儿。评估了分娩方式、血友病的类型和严重程度、出血的发作和时间、出血部位、凝血因子替代疗法的预防措施，以及联邦政府资助的血友病治疗中心 (HTC) 在管理这些血友病婴儿中所发挥的作用。 75% 的血友病婴儿在出生后第一个月就被早期诊断出来，尤其是那些有家族史或母亲是已知血友病携带者的婴儿；母亲携带者的婴儿更有可能通过剖腹产分娩。分娩前 HTC 的介入可以避免使用真空吸引器和产钳辅助分娩。包皮环切部位出血是最常见的出血并发症，其次是颅内和颅外出血以及足跟采血出血。 8% 的婴儿在出生后 24 小时内接受了浓缩因子注射；超过一半的人接受了预防出血的治疗。这项研究强调了血友病幼儿特有的初始出血率和部位，并强调需要进行研究以确定最佳的基于证据的治疗建议。
Lack of detailed natural history and outcomes data for neonates and toddlers with haemophilia hampers the provision of optimal management of the disorder. We report an analysis of prospective data collected from 580 neonates and toddlers aged 0-2 years with haemophilia enrolled in the Universal Data Collection (UDC) surveillance project of the Centers for Disease Control and Prevention (CDC). This study focuses on a cohort of babies with haemophilia whose diagnosis was established before the age of two. The mode of delivery, type and severity of haemophilia, onset and timing of haemorrhages, site(s) of bleeding, provision of prophylaxis with coagulation factor replacement therapy, and the role played by the federally funded Haemophilia Treatment Centers (HTC) in the management of these infants with haemophilia were evaluated. Seventy-five per cent of haemophilic infants were diagnosed early, in the first month of life, especially those with a family history or whose mothers were known carriers; infants of maternal carriers were more likely to be delivered by C-section. Involvement of an HTC prior to delivery resulted in avoidance of the use of assisted deliveries with vacuum and forceps. Bleeding from the circumcision site was the most common haemorrhagic complication, followed by intra- and extra-cranial haemorrhages and bleeding from heel stick blood sampling. Eight per cent of the infants were administered factor concentrate within 24 h of birth; more than half were treated to prevent bleeding. This study highlights the significant rate and the sites of initial bleeding unique to very young children with haemophilia and underscores the need for research to identify optimal evidence-based recommendations for their management.