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Clin Genet. 2001 Nov;60(5):360-5. Related Articles,Links

Evaluation for sleep apnea in patients with Ehlers-Danlos syndrome and

Marfan: a questionnaire study.

Verbraecken J, Declerck A, Van de Heyning P, De Backer W, Wouters EF.

Department of Pulmonary Medicine, University of Antwerp, UIA, Belgium.

Sleep complaints are frequently reported by patients with Marfan and

Ehlers-Danlos syndrome (EDS). We examined the exact nature of sleep complaints

in

these patients. A representative sample of Marfan and EDS patients responded to

a

general sleep questionnaire, including the Epworth Sleepiness Scale (ESS) and

the Medical Outcomes Study Short-Form 36 (SF-36) health-related quality of

life (QOL) questionnaire. Fifteen Marfan patients and 9 EDS patients were

evaluated and compared to 24 healthy controls, matched for age, sex and body

mass

index. Maintaining sleep was frequently disturbed in Marfan (40%, p < 0.04) as

well as in EDS patients (56%, p < 0.01). Sleep apnea was exclusively reported by

Marfan patients (27%, p = 0.03). Periodic limb movements were much more

reported in EDS (67%, p = 0.02) than in Marfan (27%, p = 0.25) compared to

controls

(8%). Pain and back complaints were highly presented in both groups, but most

pronounced in EDS patients (47% in Marfan versus 77% in EDS). No differences

for the scores in the ESS were found. For all SF-36 questionnaire items,

scores were much lower in patient groups, except for emotional problems. We

found

that sleep complaints were not rare in Marfan and EDS patients and correlated

well with different QOL items. Our study calls for greater attention to the

presence of apnea, pain and periodic limb movements in these patients.

PMID: 11903337 [PubMed - indexed for MEDLINE]

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