[Joint status after prophylactic substitution. An investigation in hemophilic children and adolescents in the DDR].

T Zimmer, G Weissbach, J Wendisch
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Abstract

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.

预防替代后的关节状态。德意志民主共和国血友病儿童和青少年的调查[j]。
本文分析了1987 ~ 1989年参加夏令营的98例患有严重或中度血友病的儿童和青少年的关节状况。这是由中心的报告完成的,另一方面,通过世界血友病联合会推荐的评分系统对患者进行测试。根据这些中心的报告,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,更多的患者受到慢性关节病的离散症状的影响。研究发现,两个中心之间存在巨大差异,患者的得分随着年龄的增长而增加。在一年内发现发现的缺陷并不罕见。血友病患者的关节问题仅靠预防性治疗而不能完全解决。缺乏对关节的复杂评估,预防性治疗的管理以及对预防的调整导致可避免的损害,使患者在晚年致残。
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