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

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

study id #: NCT02712060

condition: Ehler's Danlos Syndrome, Obstructive Sleep Apnea

status: completed

purpose:

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.

start date: March 2016

estimated completion: May 2017

last updated: May 15, 2017

phase of development: N/A

size / enrollment: 50

study design: Observational Model: Case Control
Time Perspective: Prospective

study 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.

primary outcomes:

  • Prevalence of OSA in children and adolescents with EDS [ Time Frame:up to 12 months ]

secondary outcomes:

  • Quality of life in children and adolescents with EDS [ Time Frame:up to 12 months ]

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

sponsor: University of Zurich

contacts: Malcolm Kohler, MD +41 44 255 3828 malcolm.kohler@usz.ch
Anna Stöberl, MD +41 44 255 1743 annasophie.stoeberl@usz.ch

investigators: Malcolm Kohler, MD University of Zurich

locations: Switzerland

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