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A group of patients with Marfan's syndrome, who have finger and toe contractures, displays tendons' alterations upon an ultrasound examination: are these features common among classical Marfan patients? | LitMetric

AI Article Synopsis

  • The study explores the musculoskeletal characteristics of patients with Marfan's syndrome, focusing on hand and foot contractures as clinical indicators of the condition.
  • Thirteen diagnosed patients underwent thorough examinations, including assessments of muscle pain, strength, joint hypermobility, and ultrasound imaging of tendons.
  • Results indicated that while tendon thickness was reduced and tendon echo-intensity showed varying textures in Marfan patients, muscle pain and hypermobility scores remained normal, suggesting structural changes in connective tissue associated with contractures.

Article Abstract

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.

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Source
http://dx.doi.org/10.1007/s11739-016-1399-5DOI Listing

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