Obstructive Sleep Apnoea in Children and Adolescents with Ehlers-Danlos Syndrome.
Ehlers-Danlos syndrome
Obstructive sleep apnoea
Paediatric patients
Rare diseases
Sleep disorders
Journal
Respiration; international review of thoracic diseases
ISSN: 1423-0356
Titre abrégé: Respiration
Pays: Switzerland
ID NLM: 0137356
Informations de publication
Date de publication:
Historique:
received:
25
07
2018
accepted:
05
10
2018
pubmed:
30
11
2018
medline:
22
8
2020
entrez:
29
11
2018
Statut:
ppublish
Résumé
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. This prospective cross-sectional study aimed at determining the prevalence of OSA in paediatric EDS patients. 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. 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). OSA is a previously underestimated EDS-related complication increasing disease burden.
Sections du résumé
BACKGROUND
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
OBJECTIVE
This prospective cross-sectional study aimed at determining the prevalence of OSA in paediatric EDS patients.
METHODS
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
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
CONCLUSIONS
OSA is a previously underestimated EDS-related complication increasing disease burden.
Identifiants
pubmed: 30485858
pii: 000494328
doi: 10.1159/000494328
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
284-291Informations de copyright
© 2018 S. Karger AG, Basel.