Obstructive Sleep Apnoea in Children and Adolescents With Ehlers-Danlos Syndrome

Completed

• Conditions: Obstructive Sleep Apnea; Ehler's Danlos Syndrome
• Interventions: Other: No intervention

• Registration Number: NCT02712060
• Lead Sponsor: University of Zurich

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.

Study Timeline

• Study Start: 2016-03
• Study First Submitted: 2016-03-14
• Study First Submitted QC: 2016-03-14
• Study First Posted: 2016-03-17
• Status Verified: 2017-05
• Primary Completion: 2017-05
• Study Completion: 2017-05
• Last Update Submitted: 2017-05-12
• Last Update Posted: 2017-05-15

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 50

Inclusion Criteria

• Informed consent
• Diagnosis of Ehlers-Danlos Syndrome (not for control group)

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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

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Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
controls	No intervention	Patients/Subjects without the diagnosis of Ehlers-Danlos syndrome
EDS patients	No intervention	Patients with the diagnosis of Ehlers-Danlos syndrome

Primary Outcome Measures

Name	Time	Method
Prevalence of OSA in children and adolescents with EDS	up to 12 months

Secondary Outcome Measures

Name	Time	Method
Quality of life in children and adolescents with EDS	up to 12 months

Trial Locations

Locations (1)

Division of Pulmonology, University Hospital Zurich; 🇨🇭 Zurich, Switzerland