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[血友病性关节病。关于51例的报道]

[Hemophilic arthropathies. Apropos of 51 cases].

作者信息

Minckes O, Rialland X, Laumonnier F, Fressinaud E, Atanasiu J P, Larget-Piet L

出版信息

Ann Pediatr (Paris). 1989 Sep;36(7):425-34.

PMID:2817697
Abstract

After defining the clinical, roentgenological and anatomic features of the various kinds of hemophilia-related joint disease (acute hemarthrosis, subacute arthritis, and chronic joint disease), we present a study of outcomes in fifty-one hemophiliac children aged 0 to 15 years and followed-up from January 1968 through December 1987 at the Angers Regional University Hospital. Four hundred and sixty-four cases of hemarthrosis were seen. Risk factors for hemarthrosis were severe hemophilia and age between 5 and 15 years, and the joints most often involved were the ankles, knees, and elbows. Sequelae of hemarthrosis were extremely prevalent in this study population: 100% of patients with severe hemophilia and 90% of patients with a factor activity of 3% or less exhibited chronic joint disease by the age of fifteen, with varying degrees of functional impairment. Because a first episode of hemarthrosis is often followed by recurrences in the same joint, we underline the need for prevention and careful treatment of acute episodes, which are the only means for decreasing articular sequelae.

摘要

在明确了各类血友病相关关节疾病(急性关节积血、亚急性关节炎和慢性关节疾病)的临床、放射学及解剖学特征后,我们对51名年龄在0至15岁之间、于1968年1月至1987年12月在昂热地区大学医院接受随访的血友病患儿的治疗结果进行了研究。共观察到464例关节积血病例。关节积血的危险因素为重度血友病以及年龄在5至15岁之间,最常受累的关节为踝关节、膝关节和肘关节。在该研究人群中,关节积血的后遗症极为普遍:到15岁时,100%的重度血友病患者和90%的因子活性为3%或更低的患者出现了慢性关节疾病,并伴有不同程度的功能障碍。由于首次关节积血发作后同一关节常复发,我们强调预防和认真治疗急性发作的必要性,这是减少关节后遗症的唯一方法。

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