Zimmer T, Weissbach G, Wendisch J
Klinik für Kinderheilkunde, Medizinischen Akademie, Carl Gustav Carus, Dresden.
Folia Haematol Int Mag Klin Morphol Blutforsch. 1990;117(4):491-8.
The joint condition in 98 children and adolescents with severe or moderate types of haemophilia participating in a summer camp between 1987 and 1989 had been analyzed. This was done by the centers' report and on the other hand with testing the patients by aid of the score system recommended by the World Federation of Hemophilia. According to the centers' reports more than 50% of all patients seem to be free of joint contractures. But according to the more sensitive score system more patients are affected by discrete symptoms of the chronic arthropathy. There had been discovered big differences between the centers and an increase of the patient's score by age. Not seldom an impairment of the findings within a single year was recognized. The joint problems in haemophiliacs are solved by the prophylactic treatment in an incomplete manner only. Lacks in the sophisticated evaluation of joints, with the management of prophylactic treatment as well as adjustment to prevention lead to avoidable damage crippling the patient on later age.
对1987年至1989年间参加一个夏令营的98名患有重度或中度血友病的儿童及青少年的关节状况进行了分析。一方面通过各中心的报告，另一方面借助世界血友病联盟推荐的评分系统对患者进行检测。根据各中心的报告，超过50%的患者似乎没有关节挛缩。但根据更敏感的评分系统，更多患者受到慢性关节病离散症状的影响。各中心之间存在很大差异，且患者评分随年龄增加。一年内检查结果出现损害的情况并不少见。血友病患者的关节问题仅通过预防性治疗得到不完全解决。在关节的精细评估、预防性治疗的管理以及预防调整方面存在不足，导致可避免的损害，使患者在晚年致残。
