title: 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?
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.
authors.affiliation: Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, VialePieraccini, 18, 50139, Florence, Italy. daniela.melchiorre@unifi.it.
authors.forename: Daniela
authors.initials: D
authors.lastname: Melchiorre
authors.affiliation: Agenzia Recupero e Riabilitazione, Careggi Hospital, University of Florence, Florence, Italy.|Department of Heart and Vessels, Regional Marfan Syndrome and Related Disorders Center, Careggi Hospital, Florence, Italy.
authors.forename: Elisa
authors.initials: E
authors.lastname: Pratelli
authors.affiliation: Department of Heart and Vessels, Regional Marfan Syndrome and Related Disorders Center, Careggi Hospital, Florence, Italy.
authors.forename: Elena
authors.initials: E
authors.lastname: Torricelli
authors.affiliation: Department of Heart and Vessels, Regional Marfan Syndrome and Related Disorders Center, Careggi Hospital, Florence, Italy.|Section of Critical Medical Care and Medical Specialities, Department of Experimental and Clinical Medicine, DENOTHE Center, University of Florence, Florence, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Sofi
authors.affiliation: Department of Heart and Vessels, Regional Marfan Syndrome and Related Disorders Center, Careggi Hospital, Florence, Italy.|Section of Critical Medical Care and Medical Specialities, Department of Experimental and Clinical Medicine, DENOTHE Center, University of Florence, Florence, Italy.
authors.forename: Rosanna
authors.initials: R
authors.lastname: Abbate
authors.affiliation: Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, VialePieraccini, 18, 50139, Florence, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Matucci-Cerinic
authors.affiliation: Department of Heart and Vessels, Regional Marfan Syndrome and Related Disorders Center, Careggi Hospital, Florence, Italy.|Section of Critical Medical Care and Medical Specialities, Department of Experimental and Clinical Medicine, DENOTHE Center, University of Florence, Florence, Italy.|Maria Agli Ulivi Center, Fondazione Don Carlo Gnocchi, Onlus, IRCCS, Florence, Italy.
authors.forename: GianFranco
authors.initials: G
authors.lastname: Gensini
authors.affiliation: Department of Heart and Vessels, Regional Marfan Syndrome and Related Disorders Center, Careggi Hospital, Florence, Italy.|Section of Critical Medical Care and Medical Specialities, Department of Experimental and Clinical Medicine, DENOTHE Center, University of Florence, Florence, Italy.
authors.forename: Guglielmina
authors.initials: G
authors.lastname: Pepe
mesh_terms: D000328:Adult; D000368:Aged; D003286:Contracture / Q000209:etiology*; D005260:Female; D005385:Fingers / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications*; D008875:Middle Aged; D010147:Pain Measurement / Q000295:instrumentation / Q000379:methods; D013710:Tendons / Q000503:physiopathology; D014034:Toes / Q000503:physiopathology*; D014463:Ultrasonography / Q000379:methods*
keywords: Contractures; Marfan’s syndrome; Tendons; Ultrasound imaging