PMID: 18198805Jan 18, 2008Paper

Assessment of sleep and breathing in adults with prader-willi syndrome: a case control series

Journal of Clinical Sleep Medicine : JCSM : Official Publication of the American Academy of Sleep Medicine
Brendon J YeeRonald R Grunstein

Abstract

Prader-Willi syndrome (PWS) is a genetic disorder (linked to chromosome 15q11-13) characterized by hypotonia and developmental delay, hyperphagia and obesity, hypersomnia and abnormal sleep, and behavioral problems. Such patients may also be at increased risk of obstructive sleep apnea (OSA), although whether this risk is explained by known risk factors has not previously been directly tested. Our aim was to compare sleep and breathing in an older group of patients with Prader-Willi syndrome with a control group-matched on the basis of age, sex, and body mass index (BMI)-in order to determine which specific features are not explained by these known confounders. Consecutive patients with PWS attending the PWS clinic at Royal Prince Alfred Hospital Sydney, Australia, were recruited. Age-, sex-, and BMI-matched controls were selected from the Sleep Investigation Unit at Royal Prince Alfred Hospital, and polysomnography-derived sleep and other parameters were compared across the groups. Nineteen subjects with PWS (14 males) were included in the study. Eighteen (95%) had a total respiratory disturbance index (TRDI) of greater than 5 events per hour, with 4 (21%) having severe obstructive sleep apnea (TRDI > or = 30 events/hour) and ...Continue Reading

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