Ghosh K
Institute of Immunohaematology, KEM Hospital, Parel, Mumbai, India.
Clin Lab Haematol. 2004 Aug;26(4):243-51. doi: 10.1111/j.1365-2257.2004.00590.x.
Eighty per cent of people with haemophilia live in developing countries, where technical expertise and health care facilities may be less than optimal. Haemophilia is a relatively rare disease and high-cost, technology-intensive therapy is not a high priority for the governments of developing countries. The rapid spread of transfusion-related viral infections in many developing countries presents further problems for haemophiliacs. However, it is possible to manage haemophiliacs patients with limited resources. Strategies for conserving factor concentrates, include education of doctors and patients, prenatal diagnosis, increasing the use of anti-fibrinolytic agents, physiotherapy, the use of fibrin glue, and simple orthotics and prosthetic measures. These approaches are helpful in the majority of these patients. Meanwhile, with the help of the World Federation of Haemophilia (WFH), all developing countries are gradually improving management skills for this relatively rare but disabling disease. The present review broadly describes the management of various aspects of severe haemophilia in developing countries.
80%的血友病患者生活在发展中国家,这些国家的技术专长和医疗保健设施可能不尽如人意。血友病是一种相对罕见的疾病,高成本、技术密集型治疗并非发展中国家政府的优先事项。在许多发展中国家,输血相关病毒感染的迅速传播给血友病患者带来了更多问题。然而,利用有限的资源也可以对血友病患者进行管理。节约凝血因子浓缩剂的策略包括对医生和患者进行教育、产前诊断、增加抗纤溶药物的使用、物理治疗、使用纤维蛋白胶以及简单的矫形和假肢措施。这些方法对大多数此类患者都有帮助。同时,在世界血友病联盟(WFH)的帮助下,所有发展中国家都在逐步提高对这种相对罕见但致残性疾病的管理技能。本综述广泛描述了发展中国家严重血友病各方面的管理情况。