📚 Volume 25, Issue 7 📋 ID: 9OYb0oD

Authors

Mateus Marino Lamari, Neuseli Marino Lamari, Michael Peres de Medeiros, Elias Naim Kassis, Idiberto Jose Zotarelli Filho, Erika Cristina Pavarino

Medicine School of São José do Rio Preto -FAMERP, São Paulo, Brazil.

Abstract

Introduction: Articular hypermobility (AH) represents the extreme range of the normal spectrum of range of motion or hereditary connective tissue disorders, with multifactorial influence, erroneously considered by most to be harmless. It occurs between 2-57% in different populations and may be associated with systemic manifestations, including maxillofacial and otolaryngological manifestations associated with AH in several studies. Hypermobility Spectrum Disorders, a group of AH-related, diagnostic exclusion conditions distinguishable from hypermobile Ehlers-Danlos Syndrome, the most common form among the 13 subtypes of the Ehlers-Danlos Syndrome, a genetically modified group heterogeneous connective tissue diseases characterized by AH, with multisystem manifestations and clinical variability, which can cause health damage, but are neglected and rarely identified. Objective: To verify the frequency of AH in the total sample and in each of the variables; characterize the corporal abilities, identify complaint of pain and of maxillofacial and otorhinolaryngological manifestations. Methods: A cross-sectional study with 57 children and adolescents in care at the Outpatient Clinic of AH and EDS of the Base Hospital of São José do Rio Preto. The data were collected from May 2016 to February 2017, with records in a questionnaire. We performed analyzes based on Beighton et al (1973) to characterize AH, with descriptive statistical analysis based on calculations of central tendency, dispersion and frequency counts in the SPSS Software version 24. Results: Children and adolescents with AH and Beighton score ≥6 accounted for just under half of the sample and filled the variables of the score for AH according to the new classification, with predominance in the upper limbs. Body skills most often were the ability to sit in the \"W\" position and to use atypical pen support for writing. Among the most prevalent clinical manifestations are pain of any location, referred by 68.4%, and buccomaxillofacial and otolaryngological signs and symptoms accounted for 84.2%. Conclusion: Children and adolescents with AH accounted for just under half of the sample and filled the variables of the AH score, with predominance in the upper limbs. The ability to sit in the \"W\" position and to use atypical pen support for writing are more frequent bodily abilities. Clinical and otorhinolaryngologic signs and symptoms were frequent. Characterizing children and adolescents with AH and early identification of signs and symptoms and other clinical manifestations is relevant for early intervention, as well as population studies to verify the impact of these manifestations in childhood.
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📝 How to Cite

Mateus Marino Lamari, Neuseli Marino Lamari, Michael Peres de Medeiros, Elias Naim Kassis, Idiberto Jose Zotarelli Filho, Erika Cristina Pavarino (2018). "Observational Study Shows The Major Considerations of Face Manifestations, Oral Health, Body Skills and Pain in Children and Adolescents With Ehlers-Danlos Syndrome". Wulfenia, 25(7).