Haemophilia Centre, Leicester Royal Infirmary, Leicester, UK.
Anaesthesia. 2015 Jan;70 Suppl 1:112-20, e39-40. doi: 10.1111/anae.12899.
It is estimated that up to 1% of the general population has a congenital bleeding disorder. With this level of disease burden, it is more likely than not that any practising surgeon or anaesthetist will, at one time or another, have occasion to manage one such patient. Congenital haemophilia, both A and B, von Willebrand's disease, and inherited qualitative platelet defects, constitute the bulk of these disorders, with the rest distributed between much rarer conditions. Although looking after such patients will continue to pose a challenge to anaesthetists, recent and continuing advances in haemostatic products, coupled with increasing awareness of haemostatic care, means that surgery in this challenging group of patients is safer now than ever before, and can now be undertaken with a degree of confidence not possible even two decades ago. Central to these recent successes has been the continuing evolution of specialised healthcare services; in particular, Haemophilia Comprehensive Care Centres. Of equal importance, at least in developed countries, has been the ease of access to highly purified, safe and effective haemostatic products. The key to successful surgical management of the patient with a bleeding disorder is a multidisciplinary approach involving not only surgeons, anaesthetists and haematologists, but also laboratory scientists, specialist physiotherapists and haemophilia nurses. With careful planning, most surgical and invasive procedures can be carried out safely in persons with haemophilia and other bleeding disorders.
据估计,高达 1%的普通人群患有先天性出血性疾病。由于这种疾病的负担,任何执业外科医生或麻醉师都有可能在某个时候需要管理这样的患者。先天性血友病 A 和 B、血管性血友病和遗传性血小板功能缺陷构成了这些疾病的大部分,其余的则分布在更为罕见的疾病中。尽管照顾这些患者对麻醉师来说仍将是一个挑战,但最近和持续的止血产品的进步,加上对止血护理的认识不断提高,意味着现在手术治疗这群具有挑战性的患者比以往任何时候都更安全,并且现在可以在一定程度上进行信心甚至在二十年前是不可能的。这些近期成功的关键是专门医疗保健服务的不断发展;特别是血友病综合护理中心。同样重要的是,至少在发达国家,易于获得高度纯化、安全有效的止血产品。成功管理出血性疾病患者的手术管理的关键是多学科方法,不仅涉及外科医生、麻醉师和血液科医生,还涉及实验室科学家、专业物理治疗师和血友病护士。通过精心规划,大多数患有血友病和其他出血性疾病的患者都可以安全地进行手术和侵入性程序。