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下肢及踝关节多发性遗传性骨软骨瘤病的自然病史。

Natural history of multiple hereditary osteochondromatosis of the lower extremity and ankle.

作者信息

Noonan Kenneth J, Feinberg Judy R, Levenda Anthony, Snead James, Wurtz L Daniel

机构信息

K4/732 Clinical Science Center, University of Wisconsin Hospitals and Clinics, 600 Highland Avenue, Madison, WI 63792-7375, U.S.A.

出版信息

J Pediatr Orthop. 2002 Jan-Feb;22(1):120-4.

PMID:11744867
Abstract

In this study the authors evaluated the natural history of the ankle joint in patients with multiple hereditary osteochondromatosis. Thirty-eight subjects with an average age of 42 years completed a detailed subjective questionnaire and underwent clinical and radiographic evaluation of their ankles. Three subjects (8%) indicated their ankle involvement affected their vocation, and 12 (32%) were limited in recreational sports. Seven patients (18%) had pain in at least one ankle on a weekly basis, with an average ankle pain score of 2.2. Ankle range of motion averaged 50 degrees and subtalar motion was considered normal in two thirds of ankles. Radiographic evaluation documented an average tibiotalar tilt of 9 degrees of ankle valgus, with evidence of degenerative joint disease noted in 14 ankles (19%). Those with arthritic changes had significantly more tibiotalar tilt and diminished ankle range of motion compared with those without radiographic signs of osteoarthritis. These findings document measurable decreases in ankle function and suggest that correction or prevention of excessive tibiotalar tilt may be warranted to improve outcome.

摘要

在本研究中,作者评估了多发性遗传性骨软骨瘤病患者踝关节的自然病史。38名平均年龄为42岁的受试者完成了一份详细的主观问卷,并接受了踝关节的临床和影像学评估。3名受试者(8%)表示踝关节受累影响了他们的职业,12名(32%)在休闲运动方面受到限制。7名患者(18%)每周至少有一个踝关节疼痛,踝关节平均疼痛评分为2.2分。踝关节活动范围平均为50度,三分之二的踝关节距下关节活动被认为正常。影像学评估显示,踝关节平均内翻9度,14个踝关节(19%)有退行性关节病迹象。与没有骨关节炎影像学表现的患者相比,有关节炎改变的患者胫距倾斜明显更大,踝关节活动范围减小。这些发现证明踝关节功能有可测量的下降,并表明矫正或预防过度的胫距倾斜可能有助于改善预后。

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