Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Medicine and Medical Specialities, Milan, Italy.
Haemophilia. 2010 Jan;16 Suppl 1:25-8. doi: 10.1111/j.1365-2516.2009.02158.x.
Treatment for children with severe haemophilia is based on prophylaxis and, if inhibitors occur, on immune tolerance induction (ITI). Both regimens require frequent infusions at early ages and therefore an adequate venous access is essential. Peripheral veins represent the best option; however, central venous catheters (CVCs) have been used to facilitate regular treatment. Unfortunately, survival of CVCs is affected by infectious and/or thrombotic complications that often lead to premature removal and consequent treatment discontinuation. This aspect may have an impact on treatment outcome, especially in the case of ITI. In light of this, internal arteriovenous fistula (AVF) has been proposed as an alternative option because of a lower rate of infectious complications. Moreover, AVF is easy to use in the home setting and is well accepted by children and parents. The possible complications are postoperative haematoma and transient symptoms of distal ischaemia; one case of symptomatic thrombosis has been reported to date. Other complications include loss of patency, aneurysmatic dilatation and limb dysmetria. A regular follow-up is mandatory to allow early remedial interventions. Surgical AVF dismantlement is recommended as soon as transition to peripheral vein access is possible.
儿童重度血友病的治疗基于预防,如果出现抑制剂,则基于免疫耐受诱导(ITI)。这两种方案都需要在早期频繁输注,因此需要有足够的静脉通路。外周静脉是最佳选择;但是,中心静脉导管(CVC)已被用于方便常规治疗。不幸的是,CVC 的存活率受到感染和/或血栓并发症的影响,这通常导致导管过早移除和随后的治疗中断。这一方面可能会对治疗结果产生影响,尤其是在 ITI 的情况下。鉴于此,已经提出了内瘘(AVF)作为替代方案,因为其感染并发症的发生率较低。此外,AVF 易于在家庭环境中使用,并且深受儿童和家长的欢迎。可能的并发症包括术后血肿和短暂的远端缺血症状;迄今为止,报告了一例有症状的血栓形成病例。其他并发症包括通畅性丧失、动脉瘤样扩张和肢体失配。为了允许早期补救干预,必须进行定期随访。一旦可以进行外周静脉通路,就推荐进行手术 AVF 拆除。