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Stöberl, AS; Gaisl, T; Giunta, C; Sievi, NA; Singer, F; Möller, A; Rohrbach, M; Kohler, M.
Obstructive Sleep Apnoea in Children and Adolescents with Ehlers-Danlos Syndrome.
Respiration. 2019; 97(4): 284-291. Doi: 10.1159/000494328
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Co-Autor*innen der Med Uni Graz
Singer Florian
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Abstract:
BACKGROUND: In Ehlers-Danlos syndrome (EDS), a group of monogenic disorders affecting connective tissues, obstructive sleep apnoea (OSA) is highly prevalent in adults. The prevalence of OSA in children with EDS is unknown. OBJECTIVES: This prospective cross-sectional study aimed at determining the prevalence of OSA in paediatric EDS patients. METHODS: Children with EDS (n = 24) were recruited from the Children's Hospital Zurich and matched to healthy controls. Participants completed home respiratory polygraphy and questionnaires (Sleep-Related Breathing Disorder Scale [SRBD], Epworth Sleepiness Scale [ESS], and Child Health Questionnaire [CHQ]). The American Academy of Sleep Medicine criteria were applied for OSA diagnosis (obstructive apnoea-hypopnoea index [oAHI] ≥1/h). Conditional logistic regression was used to compare the prevalence of OSA and to adjust for possible confounding. RESULTS: OSA was found in 42% of paediatric EDS patients and in 13% of matched controls (OR = 4.5, 95% CI = 0.97-20.83, p = 0.054). The median oAHI was higher in EDS patients than in controls (0.77/h, IQR = 0.19-1.76, vs. 0.24/h, IQR = 0.0-0.60, p < 0.001 adjusted for age, sex, and BMI z-score). EDS patients had lower scores in most CHQ scales and higher SRBD and ESS scores than controls (0.26, IQR = 0.1-0.35, vs. 0.07, IQR = 0-0.19, p = 0.004); 7 ± 4 vs. 5 ± 4, p = 0.033, respectively). CONCLUSION: OSA is a previously underestimated EDS-related complication increasing disease burden.
Find related publications in this database (using NLM MeSH Indexing)
Adolescent - administration & dosage
Case-Control Studies - administration & dosage
Child - administration & dosage
Ehlers-Danlos Syndrome - complications
Female - administration & dosage
Humans - administration & dosage
Male - administration & dosage
Prevalence - administration & dosage
Prospective Studies - administration & dosage
Quality of Life - administration & dosage
Sleep Apnea, Obstructive - epidemiology, etiology
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Find related publications in this database (Keywords)
Obstructive sleep apnoea
Sleep disorders
Rare diseases
Ehlers-Danlos syndrome
Paediatric patients
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