The Incidence and Clinical Characteristics of the Connective Tissue Dysplasia in Adolescents

Abstract

Early detection of the connective tissue dysplasia (CTD) is hampered as the used diagnostic criteria are complex and subjective. The aim of the study is to examine the incidence and clinical profile of the adolescent CTD by means of the screening checklist. Methods. The study included the pupils at the age of 10-14 years. The questionnaire designed to reveal the 7 CTD's signs (thin skin, skin hyperelasticity, keloid cicatrix, blue sclera, soft auricles, arachnodactylia, joints hypermobility), containing 12 illustrated questions with explanation for the parents, was performed as a screening method. The conditionally healthy group (the absence of CTD) consisted of the adolescents with below 12 points total and CTD group was presented by the adolescents with 13 and more points. The physical development was appreciated according to the centile tables, Quetelet index and Vargui index. The health status analysis was based on the outpatient medical records. Results. 1560 pupils were the participants of the study: 752 boys (48.2%) and 808 girls (51.8%). CTD signs were observed in 965 (61.9%) children. The adolescents with CTD's signs demonstrated the following characters more often than the adolescents without CTD's signs: soft auricles-788 (81.7%) against 277 (46.6%), skin hyperelasticity-685 (71%) against 93 (15.6%), joints hypermobility-665 (68.9%) against 203 (34.1%), blue sclera-665 (68.9%) against 184 (30.9%), arachnodactylia-534 (55.3%) against 57 (9.6%). The disharmonious physical development because of the underweight body took place in 430 (52.3%) adolescents with CTD. Compared to the conditionally healthy group the most of the adolescents with CTD had the pathology of the musculoskeletal system (scoliosis, flatfoot), myopia, vegetative-vascular dystonia, digestive apparatus diseases. The CTD is associated with greater probability of the development of above mentioned pathologies, that can be indicative of the contribution of the anomalies of the connective tissue development into the etiopathogenesis of the these diseases. Conclusion. It is established that there is the high prevalence of the CTD signs among the adolescents (pupils) at the age of 10-14 years.

Benign Joint Hypermobility Syndrome in Clinical Practice

“…Таким образом, проявления СГМС отличаются широким полиморфизмом, что значительно осложняет их унификацию, объясняет отсутствие четких классификационных критериев и рекомендаций по лечению таких пациентов, требуя активного изучения. Следует помнить о том, что наличие СГМС обуславливает не только суставный синдром, но и значительно повышает риск развития нарушений других органов и систем, в том числе сердечно-сосудистой, дыхательной, иммунной, пищеварительной, мочевыделительной и нервной [14][15][16][17]. Особой чертой является также и то, что при рождении признаки вероятной патологии не выражены, что затрудняет раннее выявление и наблюдение за такими пациентами.…”