Melchiorre Daniela, Pratelli Elisa, Torricelli Elena, Sofi Francesco, Abbate Rosanna, Matucci-Cerinic Marco, Gensini GianFranco, Pepe Guglielmina
Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, VialePieraccini, 18, 50139, Florence, Italy.
Agenzia Recupero e Riabilitazione, Careggi Hospital, University of Florence, Florence, Italy.
Intern Emerg Med. 2016 Aug;11(5):703-11. doi: 10.1007/s11739-016-1399-5. Epub 2016 Feb 22.
The involvement of the musculoskeletal system with other mild pleiotropic manifestations represents a clinical criterion, called "systemic features," to d iagnose Marfan's syndrome. We aimed to investigate the features of the hands and feet redressable contractures present in a group of Marfan patients. In 13 patients with previously diagnosed Marfan's syndrome, an accurate clinical examination was performed. In particular the characterization of the musculoskeletal system by visual analogic scale to measure muscle pain (VAS) and muscle strength (MRC system) was carried out; the Beighton scale score was used to evaluate the articular hypermobility. Ultrasound examination (US) was performed to detect deep-superficial flexor tendons and extensor tendons of both hands, and the short and long flexor and extensor tendons of the fingers and toes in static and dynamic positions. The ImageJ program was adopted to measure a profile of tendon echo-intensity. A reduction of the thickness of all tendons was detected by US in our patients; the VAS and Beighton scale scores were in normal ranges. The profile of tendon echo-intensity showed different textural details in all Marfan patients. This study provides evidence for other contractures' localization, and for altered findings of the tendons in patients with Marfan syndrome and finger/toe contractures. These changes may be associated with structural modifications in connective tissue.
肌肉骨骼系统受累并伴有其他轻度多效性表现是诊断马凡综合征的一项临床标准,称为“全身特征”。我们旨在研究一组马凡综合征患者中存在的手足可矫正挛缩的特征。对13例先前诊断为马凡综合征的患者进行了精确的临床检查。特别是通过视觉模拟量表测量肌肉疼痛(VAS)和肌肉力量(MRC系统)对肌肉骨骼系统进行了特征描述;使用Beighton量表评分来评估关节活动过度。进行超声检查(US)以检测双手的深浅屈肌腱和伸肌腱,以及手指和脚趾在静态和动态位置的短、长屈肌腱和伸肌腱。采用ImageJ程序测量肌腱回声强度轮廓。我们的患者通过超声检测到所有肌腱厚度均减少;VAS和Beighton量表评分在正常范围内。肌腱回声强度轮廓在所有马凡综合征患者中显示出不同的纹理细节。本研究为其他挛缩的定位以及马凡综合征合并手指/脚趾挛缩患者肌腱的改变提供了证据。这些变化可能与结缔组织的结构改变有关。