Pabinger I, Niessner H, Korninger C, Nowotny C, Stain M, Kyrle P A, Lechner K
I. Medizinische Universitätsklinik, Wien.
Wien Klin Wochenschr. 1987 Nov 20;99(22):773-7.
88 patients with severe haemophilia (66 with haemophilia A without inhibitor, 12 with haemophilia A and inhibitor, 10 with haemophilia B) currently receive comprehensive care at the Vienna haemophilia centre. Data available at the centre and a questionnaire answered by the hemophiliacs were used in order to evaluate the current situation of home care. At present, 62 (70%) out of 88 patients receive home treatment (51 with haemophilia A without inhibitor, 5 patients with haemophilia A and inhibitor and 6 with haemophilia B). For treatment of joint and muscle bleeding mean dosages of 15.3 units/kg body weight of factor VIII concentrate, 17.0 units/kg of factor IX concentrate and 30 units/kg of FEIBA were administered by the hemophiliacs. Children and young patients required higher doses (30 and 17.4 units/kg F VIII, respectively). Two thirds of the bleeding episodes were successfully treated by a single infusion. No severe side effects were observed during home treatment. Home treatment has been widely accepted by the patients. It is regarded as a practical and safe therapy and has improved the life quality of haemophiliac patients.
88例重度血友病患者（66例甲型血友病无抑制物，12例甲型血友病有抑制物，10例乙型血友病）目前在维也纳血友病中心接受综合治疗。为评估家庭护理的现状，使用了该中心现有的数据以及血友病患者填写的问卷。目前，88例患者中有62例（70%）接受家庭治疗（51例甲型血友病无抑制物，5例甲型血友病有抑制物，6例乙型血友病）。血友病患者治疗关节和肌肉出血时，平均剂量分别为每公斤体重15.3单位的凝血因子VIII浓缩物、17.0单位的凝血因子IX浓缩物和30单位的旁路活化剂。儿童和年轻患者需要更高剂量（分别为每公斤体重30和17.4单位的凝血因子VIII）。三分之二的出血事件通过单次输注成功治疗。家庭治疗期间未观察到严重副作用。家庭治疗已被患者广泛接受。它被视为一种实用且安全的治疗方法，提高了血友病患者的生活质量。
