Real-world data describing emicizumab prophylaxis in pediatric hemophilia A is limited with current literature providing a heterogenous approach to procedural management. We performed a retrospective review on the 28 patients who have been treated with emicizumab prophylaxis at Texas Children's Hospital from 2018 to 2021. After starting emicizumab, the mean annualized bleeding rate reduced from 3.61 to 0.44. Seven surgical procedures were performed in the cohort, and all treated with pre- and postoperative factor replacement resulting in one minor bleeding event. We demonstrate a successful experience with emicizumab prophylaxis and safe perioperative approach with a focus on minimizing postoperative bleeding.

中文翻译：

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Emicizumab 治疗小儿血友病：一项单中心回顾性研究的出血和手术结果

描述 emicizumab 预防小儿 A 型血友病的真实数据有限，目前的文献提供了一种异质的程序管理方法。我们对 2018 年至 2021 年在德克萨斯儿童医院接受 emicizumab 预防性治疗的 28 名患者进行了回顾性分析。开始使用 emicizumab 后，平均年出血率从 3.61 降至 0.44。在该队列中进行了七次手术，所有手术均在术前和术后进行因子替代治疗，导致 1 次轻微出血事件。我们展示了 emicizumab 预防和安全围手术期方法的成功经验，重点是尽量减少术后出血。