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Progression of Obstructive Sleep Apnea Syndrome in Pediatric Patients with Prader–Willi Syndrome

Obstructive sleep apnea syndrome (OSAS) is one of the most common comorbidities in patients with Prader–Willi syndrome (PWS) and causes significant consequences. This observational study was conducted to investigate the progression of OSAS in pediatric patients with PWS, who had not undergone upper...

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Autores principales: Wong, Shi-Bing, Yang, Mei-Chen, Tzeng, I-Shiang, Tsai, Wen-Hsin, Lan, Chou-Chin, Tsai, Li-Ping
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9221549/
https://www.ncbi.nlm.nih.gov/pubmed/35740849
http://dx.doi.org/10.3390/children9060912
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author Wong, Shi-Bing
Yang, Mei-Chen
Tzeng, I-Shiang
Tsai, Wen-Hsin
Lan, Chou-Chin
Tsai, Li-Ping
author_facet Wong, Shi-Bing
Yang, Mei-Chen
Tzeng, I-Shiang
Tsai, Wen-Hsin
Lan, Chou-Chin
Tsai, Li-Ping
author_sort Wong, Shi-Bing
collection PubMed
description Obstructive sleep apnea syndrome (OSAS) is one of the most common comorbidities in patients with Prader–Willi syndrome (PWS) and causes significant consequences. This observational study was conducted to investigate the progression of OSAS in pediatric patients with PWS, who had not undergone upper airway surgery, through a longitudinal follow-up of their annual polysomnography results. Annual body mass index (BMI), BMI z-score, sleep efficiency and stages, central apnea index (CAI), obstructive apnea–hypopnea index (OAHI), and oxygen saturation nadir values were longitudinally analyzed. At enrollment, of 22 patients (10 boys and 12 girls) aged 11.7 ± 3.9 years, 20 had OSAS. During the 4-year follow-up, only two patients had a spontaneous resolution of OSAS. The average BMI and BMI z-score increased gradually, but CAI and OAHI showed no significant differences. After statistical adjustment for sex, age, genotype, growth hormone use, and BMI z-score, OAHI was associated with the BMI z-score and deletion genotype. In conclusion, OSAS is common in patients with PWS, and rarely resolved spontaneously. Watchful waiting may not be the best OSAS management strategy. Weight maintenance and careful selection of surgical candidates are important for OSAS treatment in patients with PWS.
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spelling pubmed-92215492022-06-24 Progression of Obstructive Sleep Apnea Syndrome in Pediatric Patients with Prader–Willi Syndrome Wong, Shi-Bing Yang, Mei-Chen Tzeng, I-Shiang Tsai, Wen-Hsin Lan, Chou-Chin Tsai, Li-Ping Children (Basel) Article Obstructive sleep apnea syndrome (OSAS) is one of the most common comorbidities in patients with Prader–Willi syndrome (PWS) and causes significant consequences. This observational study was conducted to investigate the progression of OSAS in pediatric patients with PWS, who had not undergone upper airway surgery, through a longitudinal follow-up of their annual polysomnography results. Annual body mass index (BMI), BMI z-score, sleep efficiency and stages, central apnea index (CAI), obstructive apnea–hypopnea index (OAHI), and oxygen saturation nadir values were longitudinally analyzed. At enrollment, of 22 patients (10 boys and 12 girls) aged 11.7 ± 3.9 years, 20 had OSAS. During the 4-year follow-up, only two patients had a spontaneous resolution of OSAS. The average BMI and BMI z-score increased gradually, but CAI and OAHI showed no significant differences. After statistical adjustment for sex, age, genotype, growth hormone use, and BMI z-score, OAHI was associated with the BMI z-score and deletion genotype. In conclusion, OSAS is common in patients with PWS, and rarely resolved spontaneously. Watchful waiting may not be the best OSAS management strategy. Weight maintenance and careful selection of surgical candidates are important for OSAS treatment in patients with PWS. MDPI 2022-06-17 /pmc/articles/PMC9221549/ /pubmed/35740849 http://dx.doi.org/10.3390/children9060912 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Wong, Shi-Bing
Yang, Mei-Chen
Tzeng, I-Shiang
Tsai, Wen-Hsin
Lan, Chou-Chin
Tsai, Li-Ping
Progression of Obstructive Sleep Apnea Syndrome in Pediatric Patients with Prader–Willi Syndrome
title Progression of Obstructive Sleep Apnea Syndrome in Pediatric Patients with Prader–Willi Syndrome
title_full Progression of Obstructive Sleep Apnea Syndrome in Pediatric Patients with Prader–Willi Syndrome
title_fullStr Progression of Obstructive Sleep Apnea Syndrome in Pediatric Patients with Prader–Willi Syndrome
title_full_unstemmed Progression of Obstructive Sleep Apnea Syndrome in Pediatric Patients with Prader–Willi Syndrome
title_short Progression of Obstructive Sleep Apnea Syndrome in Pediatric Patients with Prader–Willi Syndrome
title_sort progression of obstructive sleep apnea syndrome in pediatric patients with prader–willi syndrome
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9221549/
https://www.ncbi.nlm.nih.gov/pubmed/35740849
http://dx.doi.org/10.3390/children9060912
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