Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Internal Medicine and Medical Specialties, IRCCS Maggiore Hospital, Mangiagalli and Regina Elena Foundation and University of Milan, Milan, Italy.
Haemophilia. 2010 Jan;16 Suppl 1:20-4. doi: 10.1111/j.1365-2516.2009.02156.x.
Current haemophilia treatment in children is based on regular intravenous infusions of concentrates for prolonged periods, according to prophylaxis regimens or immune tolerance induction treatment, in cases of inhibitor development. Therefore, a stable and uncomplicated venous access is required and as such peripheral veins represent the preferred option. However, frequent infusions in the home setting can be problematic in very young children and for this reason, central venous access devices (CVADs) have been widely used to improve treatment feasibility. Unfortunately CVADs' use is associated with a high rate of complications, and infections and thrombotic occlusion can influence treatment outcome by causing unwanted treatment interruption. CVADs can be grouped into three main categories: external non-tunnelled, external tunnelled and fully implantable devices known as ports. The management of CVADs at home often represents a challenge because a strict adherence to sterile procedures is required. Indeed, the incidence of infections with ports is much lower than that reported for external devices; however, ports carry the inconvenience of needle sticks. More recently, arteriovenous fistula was shown to be a suitable alternative to CVADs because it is easy to use and is associated with a lower rate of complication.
目前，儿童的血友病治疗主要依据预防方案或免疫耐受诱导治疗，通过定期静脉输注浓缩物来进行，以防止抑制剂的产生。因此，需要建立一个稳定且不复杂的静脉通路，外周静脉是首选。然而，对于非常年幼的儿童来说，家庭环境下的频繁输注可能会出现问题，因此，中心静脉通路装置（CVAD）已被广泛用于提高治疗可行性。不幸的是，CVAD 的使用与高并发症发生率相关，感染和血栓闭塞会通过导致不必要的治疗中断来影响治疗效果。CVAD 可分为三类：外部无隧道、外部隧道和完全可植入的称为端口的设备。CVAD 的家庭管理通常是一个挑战，因为需要严格遵守无菌程序。事实上，与外部设备相比，端口的感染发生率要低得多；但是，端口存在针刺的不便。最近，动静脉瘘被证明是 CVAD 的一种合适替代方法，因为它易于使用，并且并发症发生率较低。
