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To evaluate autonomic regulation in patients with Prader-Willi syndrome with sleep-disordered breathing.
To evaluate the relationship between narcolepsy and sleep-disordered breathing, the natural course of untreated or treated sleep-disordered breathing and its impacts on autonomic regulation in patients with Prader-Willi syndrome with sleep-disordered breathing.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Control | Healthy subjects, 7-75 y/o |
| |
| Study | 7-75 y/o, diagnosed with Prader-Willi syndrome. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Polysomnography | Diagnostic Test | overnight polysomnography recording at least six hours |
|
| Measure | Description | Time Frame |
|---|---|---|
| Narcolepsy prevalence | Compare the narcolepsy prevalence amount healthy and Prader-Willi syndrome with or without sleep-disordered breathing | 1day |
| Measure | Description | Time Frame |
|---|---|---|
| The natural course and its impact of untreated sleep-disordered breathing on autonomic regulation in patients with Prader-Willi syndrome. | Autonomic function measurements (low frequency power and high frequency power, presented as normalized units, and the overall autonomic regulation presented as Low frequency/high frequency ratio) using the EKG signal extracting from overnight polysomnography recording raw data among Prader-Willi patients with untreated sleep-disordered breathing at baseline and after a period of follow-up. |
| Measure | Description | Time Frame |
|---|---|---|
| The effect of continuous positive airway pressure on autonomic regulation in patients with Prader-Willi syndrome with sleep-disordered breathing. |
|
Inclusion Criteria:
Exclusion Criteria:
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7-75 y/o Prader-Willi syndrome patients or healthy subjects, can perform polysomnography, multiple sleep latency test, orthostatic methods for autonomic function test, can perform pulmonary function test.
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| 美貞 楊, MD | Contact | 0970333784 | mimimai3461@gmail.com |
| Name | Affiliation | Role |
|---|---|---|
| 美貞 楊, MD | Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Taipei Tzu-Chi Hospital, Buddhist Tzu-Chi Medical Foundation | Recruiting | New Taipei City | 23143 | Taiwan |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 29294134 | Background | Ghergan A, Coupaye M, Leu-Semenescu S, Attali V, Oppert JM, Arnulf I, Poitou C, Redolfi S. Prevalence and Phenotype of Sleep Disorders in 60 Adults With Prader-Willi Syndrome. Sleep. 2017 Dec 1;40(12). doi: 10.1093/sleep/zsx162. | |
| 24733986 | Background | Sedky K, Bennett DS, Pumariega A. Prader Willi syndrome and obstructive sleep apnea: co-occurrence in the pediatric population. J Clin Sleep Med. 2014 Apr 15;10(4):403-9. doi: 10.5664/jcsm.3616. |
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| ID | Term |
|---|---|
| D054969 | Primary Dysautonomias |
| ID | Term |
|---|---|
| D001342 | Autonomic Nervous System Diseases |
| D009422 | Nervous System Diseases |
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| ID | Term |
|---|---|
| D017286 | Polysomnography |
| D045422 | Continuous Positive Airway Pressure |
| ID | Term |
|---|---|
| D008991 | Monitoring, Physiologic |
| D019937 | Diagnostic Techniques and Procedures |
| D003933 | Diagnosis |
| D011175 | Positive-Pressure Respiration |
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| Multiple sleep latency test | Diagnostic Test | daytime naps at least four to five naps to evaluate daytime sleepiness and narcolepsy |
|
|
| Continuous positive airway pressure | Other | continuous positive airway pressure treatment for sleep-disordered breathing |
|
|
| 12 months |
| 3 months |
| 24126982 | Background | Miller J, Wagner M. Prader-Willi syndrome and sleep-disordered breathing. Pediatr Ann. 2013 Oct;42(10):200-4. doi: 10.3928/00904481-20130924-10. |
| 10607116 | Background | Clift S, Dahlitz M, Parkes JD. Sleep apnoea in the Prader-Willi syndrome. J Sleep Res. 1994 Jun;3(2):121-126. doi: 10.1111/j.1365-2869.1994.tb00115.x. |
| 26716917 | Background | Scammell TE. Narcolepsy. N Engl J Med. 2015 Dec 31;373(27):2654-62. doi: 10.1056/NEJMra1500587. No abstract available. |
| D012121 | Respiration, Artificial |
| D058109 | Airway Management |
| D013812 | Therapeutics |
| D012138 | Respiratory Therapy |