重度甲型血友病患儿中心静脉通路装置植入：来自中国儿童综合关怀中心的数据
Central venous access devices implantation in children with severe hemophilia a: data from the children comprehensive care center of China.
作者信息
Xu Qian, Wang Chunli, Cheng Wei, Zhen Yingzi, Ding Yaguang, Liu Guoqing, Yao Wanru, Chen Zhenping, Li Zhiqiang, Wu Runhui
机构信息
Department II of Hematology Center, Haemophilia Comprehensive Care Center, Beijing Children's Hospital, Capital Medical University; National Center for Children's Health, Beijing 100045, China.
Hematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, Beijing 100045, China.
出版信息
Heliyon. 2023 Feb 11;9(3):e13666. doi: 10.1016/j.heliyon.2023.e13666. eCollection 2023 Mar.
OBJECTIVES
To report the perioperative management experience of central venous access devices (CVAD) in Chinese children with severe hemophilia A (SHA) in China.
METHODS
This retrospective study included SHA children who underwent Port-A-Cath or peripherally inserted central catheter (PICC) implantation between 2020/01 and 2021/07. Collected data included baseline characteristics, factor replacement regimen and CVAD-related complications.
RESULTS
Nine patients had nine ports placed, and eight patients underwent 10 PICCs placement. Patients without or with low-titer inhibitor (<5 BU) received a port. The median preoperative and postoperative plasma-derived factor VIII (pd-FVIII) doses were 53.0 (44.4-61.1) and 315.9 (88.2-577.8) IU/kg. The median port duration was 189 (15-512) days, with infection incidence of 0.06 per 1000 CVAD days. Patients with high-titer inhibitors (>10 BU) received PICC. The median recombinant factor VIIa (rFVIIa) dose was 87.47 μg/kg before and for 5-7 doses after implantation over 2-3 days. The median PICC duration was 226.5 days, with infection incidence of 0.12 per 1000 catheter-days.
CONCLUSIONS
CVADs can be safely implanted in China. PICC implantation is a practical and safe option for SHA children with high-titer inhibitors.
目的
报告中国重型甲型血友病（SHA）患儿中心静脉通路装置（CVAD）的围手术期管理经验。
方法
这项回顾性研究纳入了2020年1月至2021年7月期间接受植入式静脉输液港或经外周静脉穿刺中心静脉置管（PICC）的SHA患儿。收集的数据包括基线特征、凝血因子替代方案和CVAD相关并发症。
结果
9例患者植入了9个静脉输液港，8例患者进行了10次PICC置管。无抑制物或低滴度抑制物（<5 BU）的患者接受了静脉输液港植入。术前和术后血浆源性凝血因子VIII（pd-FVIII）剂量的中位数分别为53.0（44.4-61.1）和315.9（88.2-577.8）IU/kg。静脉输液港的中位使用时长为189（15-512）天，每1000个CVAD日的感染发生率为0.06。高滴度抑制物（>10 BU）的患者接受了PICC置管。植入前重组凝血因子VIIa（rFVIIa）剂量的中位数为87.47 μg/kg，植入后2-3天内给予5-7剂，剂量中位数为87.47 μg/kg。PICC的中位使用时长为226.5天，每1000个导管日的感染发生率为0.12。
结论
在中国，CVAD可以安全植入。对于有高滴度抑制物的SHA患儿，PICC置管是一种实用且安全的选择。
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