Ljung R, Aronis-Vournas S, Kurnik-Auberger K, van den Berg M, Chambost H, Claeyssens S, van Geet C, Glomstein A, Hann I, Hill F, Kobelt R, Kreuz W, Mancuso G, Muntean W, Petrini P, Rosado L, Scheibel E, Siimes M, Smith O, Tusell J
Department of Paediatrics, University Hospital, SE-205 02 Malmö, Sweden.
Haemophilia. 2000 Nov;6(6):619-24. doi: 10.1046/j.1365-2516.2000.00427.x.
A survey was made of the current status of treatment of haemophilic boys at 20 centres in 16 European countries and includes approximately 1500 of the estimated 6500 haemophiliacs in the participating countries. Many mild haemophiliacs are not seen, or seen infrequently, at haemophilia centres and this requires study. Nine of 18 centres provide continuous prophylaxis to 80-100% of their patients, five centres provide it to 55-80% and the remaining four centres to 15-40% of the boys. The median dose given was 6240 U kg-1 year-1 (range 3120-7800). Four centres administered only recombinant concentrates to children with severe haemophilia A, while seven centres administered recombinant concentrates to 75-90% and the remaining centres to less than 50% of the boys (two centres < 10%). When asked for the choice of concentrate for a newly diagnosed boy with severe haemophilia A, all but one centre preferred recombinant concentrate. Most boys below 6 years received concentrates via a peripheral vein but three centres preferred a central venous line for 80-100% of the boys. Thirteen of 18 centres applied home treatment to 84-100% of the boys and the remaining five centres to 57-77% of the boys.
对16个欧洲国家20个中心的血友病男孩治疗现状进行了一项调查，涵盖了参与国估计6500名血友病患者中的约1500名。许多症状较轻的血友病患者不在血友病中心就诊，或很少就诊，这需要进行研究。18个中心中有9个为80 - 100%的患者提供持续预防治疗，5个中心为55 - 80%的患者提供，其余4个中心为15 - 40%的男孩提供。给予的中位剂量为6240 U kg-1年-1（范围3120 - 7800）。4个中心仅对重度甲型血友病儿童使用重组凝血因子浓缩剂，7个中心对75 - 90%的男孩使用重组凝血因子浓缩剂，其余中心对不到50%的男孩使用（2个中心<10%）。当被问及为新诊断的重度甲型血友病男孩选择凝血因子浓缩剂时，除一个中心外，所有中心都倾向于重组凝血因子浓缩剂。大多数6岁以下的男孩通过外周静脉接受凝血因子浓缩剂，但有3个中心80 - 100%的男孩更倾向于使用中心静脉导管。18个中心中有13个对84 - 100%的男孩采用家庭治疗，其余5个中心对57 - 77%的男孩采用家庭治疗。
