ArticleClinical autonomic research : official journal of the Clinical Autonomic Research Society2025
Cardiovascular autonomic dysfunction and sleep abnormalities in children with Prader-Willi syndrome.
Article in Clinical autonomic research : official journal of the Clinical Autonomic Research Society, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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Who cites it
2 citing papers in PubMed.
- Age Does Not Affect Respiratory Characteristics in Children With Prader-Willi Syndrome Before and After Growth Hormone Therapy.Acta paediatrica (Oslo, Norway : 1992) · 2026Article
- Autonomic Control of Heart Rate During Sleep Is Depressed in Young Children With Prader-Willi Syndrome.Journal of sleep research · 2026Article
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7 authors.
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Abstract
purposePrader-Willi syndrome (PWS) is a rare genetic neurodevelopmental condition characterized by cognitive disabilities, behavioral problems, hypothalamic dysfunction with obesity, and sleep disorders. A few studies have reported autonomic nervous system dysfunction. Our aim was to investigate dysautonomia by combining sleep studies and standard autonomic testing in regularly followed children with PWS.
methodsIn this retrospective study, heart rate variability was analyzed during each sleep stage (polysomnography) using time and frequency domains in PWS children (N = 37) compared with age-matched controls (N = 20). Cardiovascular autonomic testing (Ewing tests) and sweating assessment (electrochemical skin conductance) were also performed in patients over 6 years (N = 23).
resultsAutonomic testing: Heart rate changes with active standing and with deep breathing were impaired in 47% and 22% of the children, respectively. Asymptomatic orthostatic hypotension (OH) was found in 26%. Baroreflex sensitivity in supine position was in normal range (14.1 ± 6.7 ms/mmHg). Electrochemical skin conductance was normal. Sleep study: 46% of the children with PWS had obstructive sleep apnea and 24% had central sleep apnea. None of these events were observed in the control group. Mean R-R and time domain heart rate variability parameters were significantly lower compared with controls in N2 and Rapid Eye Movement (REM) sleep stages. Narcoleptic-like phenotype was found in 47% associated with lower low-frequency (LF) power (sympathetic index) in REM sleep.
conclusionOur study confirms a decreased vagal modulation during both wakefulness and sleep in children with PWS. OH in some patients suggests a sympathetic dysfunction. These changes may contribute to the increased cardiovascular risk in PWS.
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