Pettersson H, Nilsson I M, Hedner U, Noréhn K, Ahlberg A
Acta Paediatr Scand. 1981 Jul;70(4):565-70. doi: 10.1111/j.1651-2227.1981.tb05741.x.
The effect of prophylactic treatment of severe haemophilia A and B was evaluated with a recently described radiologic score system for assessing the severity of arthropathy. The severity of the arthropathy was assessed in 50 patients with severe haemophilia receiving prophylaxis with factor VIII or IX (25--30 units factor VIII or IX/kg bodyweight at 4--7 day intervals), and in 9 in whom the condition was complicated by inhibitors and who were thus not receiving prophylaxis. The score values found in a recent investigation of the natural course of haemophilia served as controls in the evaluation of the effect of the prophylaxis. In the group with severe haemophilia receiving prophylaxis changes appeared only exceptionally in joints not affected already before the prophylaxis, and the course of the arthropathy and its severity according to age did not vary to any noteworthy extent from that in the control group with moderate haemophilia. In the cases complicated by inhibitors the joints were most often worse than those in the control group with severe haemophilia. It is concluded that prophylaxis has a considerable effect on the course of the arthropathy and the prophylaxis should be instituted at an early stage of the disease, when the joints are still unaffected.
采用最近描述的一种用于评估关节病严重程度的放射学评分系统，对重度甲型和乙型血友病的预防性治疗效果进行了评估。对50例接受凝血因子VIII或IX预防性治疗（每4 - 7天一次，剂量为25 - 30单位凝血因子VIII或IX/千克体重）的重度血友病患者以及9例因产生抑制剂而未接受预防性治疗且病情复杂的患者的关节病严重程度进行了评估。在评估预防性治疗效果时，将最近一项关于血友病自然病程研究中得到的评分值用作对照。在接受预防性治疗的重度血友病组中，仅在预防性治疗前未受影响的关节中偶尔出现变化，并且根据年龄的关节病病程及其严重程度与中度血友病对照组相比，没有显著差异。在有抑制剂并发症的病例中，关节状况通常比重度血友病对照组更差。得出的结论是，预防性治疗对关节病病程有显著影响，并且应在疾病早期关节仍未受影响时就开始进行预防性治疗。
