Hypermobility of Joints

XVI, 204 p.online resource

A connective tissue disorder may underlie ESSENCE problems in childhood

© 2016 Elsevier Ltd Background Ehlers-Danlos syndrome hypermobility type, also known as Joint Hypermobility Syndrome (EDS-HT/JHS), is the most common hereditary disorder of the connective tissue (HDCT). It is characterized by tissue fragility, joint hypermobility and a wide range of articular and non-articular manifestations, which often appear in infancy. The clinical picture of EDS-HT/JHS is poorly known by the medical community, as is the presence of “ESSENCE” (Early Symptomatic Syndromes Eliciting Neurodevelopmental Clinical Examinations) problems in affected children. Aim The present work reviews the clinical and empirical evidence for ESSENCE difficulties in children with EDS-HT/JHS. Method A narrative review of the literature was undertaken following a comprehensive search of scientific online databases and reference lists. This included publications of quantitative and qualitative research. Results Motor abnormality, hyperactivity/hypoactivity, inattention, speech/language, so

Severe periodontitis in Marfan's syndrome: A case report

Background: Connective tissue disorders, such as some forms of Ehlers-Danlos syndrome, have been associated with severe periodontitis. Methods: This report describes a case of Marfan's syndrome, an inherited disorder of connective tissue caused by mutations in the fibrillin-1 gene, in which the patient presented with severe periodontitis. Results: At examination, an average full-mouth clinical attachment level loss of 5.6 ± 2.1 mm, furcation involvement, and severe alveolar bone loss were observed in a 41-year-old Caucasian male. Tooth hypermobility was also present. Conclusions: This case appears to be the first documentation of severe periodontitis in a patient with Marfan's syndrome. It supports the hypothesis that a variety of connective tissue disorders may confer increased susceptibility to periodontal tissue breakdown.Link_to_subscribed_fulltex