Polysomnography test and sleep disordered breathing in Prader-Willi syndrome
INTRODUCTION Prader Willi syndrome (PWS) is a rare condition and represents the most frequent cause of syndromic obesity. Sleep apnea is a life-threatening affection and is documented as the main cause of sudden death in PWS. OBJECTIVES AND BACKGROUND The aim of our study was to evaluate sleep...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Victor Babes University of Medicine and Pharmacy Timisoara
2016-12-01
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| Series: | Research and Clinical Medicine |
| Subjects: | |
| Online Access: | http://www.resclinmed.eu/public/data_files/articles/34/article_34.pdf |
| Summary: | INTRODUCTION
Prader Willi syndrome (PWS) is a rare condition and
represents the most frequent cause of syndromic obesity.
Sleep apnea is a life-threatening affection and is
documented as the main cause of sudden death in PWS.
OBJECTIVES AND BACKGROUND
The aim of our study was to evaluate sleep disorders
in PWS patients.
MATERIALS AND METHODS
We used a portable monitor that recorded time in
bed (TIB), the air flow in the upper airways, oxygen
saturation, heart rate and snoring. The included patients
had a positive clinical and molecular diagnosis of PWS.
RESULTS
The mean of TIB was 439.3±117.19 minutes. We
recorded obstructive, central and mixed apnea, hypopnea
and short wakes caused by respiratory events that were
variable number and duration, in all patients.
cardiovascular morbidity and mortality and
improved life quality. Moreover, small doses of these
drugs proved to be effective even in patients where
hemodialysis alone was enough to control blood pressure.
CONCLUSIONS
Sleep disorders are present in most PWS patients,
not only obese ones according to their anatomical
particularities. These events prevent the use of growth
hormone therapy, the only available treatment that
decreases the adipose mass and increase both prognosis
and life quality in PWS patients.
Graphical abstract: Polysomnography Test
in a PWS patient.
REFERENCES
1. Vandeleur M, Davey MJ, Nixon GM. Are sleep
studies helpful in children with Prader-Willi
syndrome prior to commencement of growth
hormone therapy? J Paediatr Child Health.
2013;49:238–41.
2. Giordano L, Toma S, Palonta F, Teggi R,
Zucconi M, Candia SD, et al. Obstructive sleep
apnea in Prader-Willi syndrome: risks and
advantages of adenotonsillectomy. Pediatr Med
Chir. 2015;37(2).
3. Pavone M, Caldarelli V, Khirani S, Colella M,
Ramirez A, Aubertin G, et al. Sleep disordered
breathing in patients with Prader-Willi syndrome:
A multicenter study. Pediatr Pulmonol.
2015;50:1354–9 |
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| ISSN: | 2360-1124 2537-5393 |