Ehler's Danlos Syndrome, Obstructive Sleep Apnea
Conditions
Keywords
Ehlers-Danlos Syndrome, Obstructive Sleep Apnea, Prevalence, Children, Pediatric, Adolescents
Brief summary
Ehlers-Danlos Syndrome (EDS) is a clinically and genetically heterogeneous group of inherited connective tissue disorders characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. EDS features such as genetically related cartilage defects, craniofacial abnormalities and increased pharyngeal collapsibility have been proposed to cause obstructive sleep apnoea (OSA). There is evidence from studies based on questionnaires that EDS patients might be more frequently affected by OSA and sleep disturbances than the general population. However, the actual prevalence of OSA in children and adolescents with EDS is unknown. The primary objective of this study is to assess the prevalence of OSA in children and adolescents with EDS (25) compared to a matched control group (25). The secondary objective of this pioneer study is to assess the quality of life in children and adolescents in EDS in comparison to healthy children and adolescents.
Detailed description
Ehlers-Danlos Syndrome (EDS) is a clinically and genetically heterogeneous group of inherited connective tissue disorders characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. EDS features such as genetically related cartilage defects, craniofacial abnormalities and increased pharyngeal collapsibility have been proposed to cause obstructive sleep apnoea (OSA). There is evidence from studies based on questionnaires that EDS patients might be more frequently affected by OSA and sleep disturbances than the general population. However, the actual prevalence of OSA in children and adolescents with EDS is unknown. The primary objective of this study is to assess the prevalence of OSA in children and adolescents with EDS compared to a matched control group. The secondary objective of this pioneer study is to assess the quality of life in children and adolescents in EDS in comparison to healthy children and adolescents.
Interventions
Sponsors
Study design
Eligibility
Inclusion criteria
* Informed consent * Diagnosis of Ehlers-Danlos Syndrome (not for control group)
Exclusion criteria
* Moribund or severe disease prohibiting protocol adherence * Continuous positive airway pressure treatment for OSA during sleep study * Physical or intellectual impairment precluding informed consent or protocol adherence * Pregnant patients
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Prevalence of OSA in children and adolescents with EDS | up to 12 months |
Secondary
| Measure | Time frame |
|---|---|
| Quality of life in children and adolescents with EDS | up to 12 months |
Countries
Switzerland