Sleep disordered breathing in Noonan syndrome: a rare case report

We report the case of a child with Noonan syndrome affected by hypertrophic cardiomyopathy and severe obstructive sleep apnea syndrome. The patient was born at 35 weeks gestational age (birth weight 3,340 g) and required mechanical ventilation. At the age of 4 years and 2 months, he was referred to...

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Main Authors: Marco Zaffanello, Antonia Maria Prioli, Luca Sacchetto, Emma Gasperi, Alfonso Bisceglia, Anna Ludovica Ghobert, Giorgio Piacentini
Format: Article
Language:English
Published: Hygeia Press di Corridori Marinella 2018-08-01
Series:Journal of Pediatric and Neonatal Individualized Medicine
Subjects:
Online Access:https://www.jpnim.com/index.php/jpnim/article/view/631
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author Marco Zaffanello
Antonia Maria Prioli
Luca Sacchetto
Emma Gasperi
Alfonso Bisceglia
Anna Ludovica Ghobert
Giorgio Piacentini
author_facet Marco Zaffanello
Antonia Maria Prioli
Luca Sacchetto
Emma Gasperi
Alfonso Bisceglia
Anna Ludovica Ghobert
Giorgio Piacentini
author_sort Marco Zaffanello
collection DOAJ
description We report the case of a child with Noonan syndrome affected by hypertrophic cardiomyopathy and severe obstructive sleep apnea syndrome. The patient was born at 35 weeks gestational age (birth weight 3,340 g) and required mechanical ventilation. At the age of 4 years and 2 months, he was referred to our center for sleep cardiorespiratory polygraphy. His parents reported frequent obstructive sleep apnea, noisy respiration and snoring, oral breathing and falling asleep during the day. They were anxious that the child could die from asphyxiation during sleep. After the polygraph study had started, the patient began napping and immediately the polygraph showed several bouts of obstructive apnea and oxygen desaturation. He was referred for urgent adenotomy. At the age of 4 years and 5 months (3 months after surgery), the symptomatology and repeat sleep polygraph study showed a marked improvement. In conclusion, obstructive sleep apnea is a very hazardous condition for children with Noonan syndrome. They are a population at risk because of facial problems and cardiopathy. The real global incidence of obstructive sleep apnea syndrome in these children is unknown.
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spelling doaj.art-edd522d286f2445d8dcf332d68a7c81c2022-12-22T01:25:36ZengHygeia Press di Corridori MarinellaJournal of Pediatric and Neonatal Individualized Medicine2281-06922018-08-0172e070225e07022510.7363/070225531Sleep disordered breathing in Noonan syndrome: a rare case reportMarco Zaffanello0Antonia Maria Prioli1Luca Sacchetto2Emma Gasperi3Alfonso Bisceglia4Anna Ludovica Ghobert5Giorgio Piacentini6Department of Surgical Sciences, Dentistry, Gynecology and Pediatrics, Pediatric Division, University of Verona, Verona, ItalyDivision of Cardiac Surgery, AOUI of Verona, Verona, ItalyDepartment of Surgical Sciences, Dentistry, Gynecology and Pediatrics, Otorhinolaryngology Section, University of Verona, Verona, ItalyDepartment of Surgical Sciences, Dentistry, Gynecology and Pediatrics, Pediatric Division, University of Verona, Verona, ItalyDepartment of Anaesthesia and Intensive Care, AOUI of Verona, Verona, ItalyDepartment of Surgical Sciences, Dentistry, Gynecology and Pediatrics, Pediatric Division, University of Verona, Verona, ItalyDepartment of Surgical Sciences, Dentistry, Gynecology and Pediatrics, Pediatric Division, University of Verona, Verona, ItalyWe report the case of a child with Noonan syndrome affected by hypertrophic cardiomyopathy and severe obstructive sleep apnea syndrome. The patient was born at 35 weeks gestational age (birth weight 3,340 g) and required mechanical ventilation. At the age of 4 years and 2 months, he was referred to our center for sleep cardiorespiratory polygraphy. His parents reported frequent obstructive sleep apnea, noisy respiration and snoring, oral breathing and falling asleep during the day. They were anxious that the child could die from asphyxiation during sleep. After the polygraph study had started, the patient began napping and immediately the polygraph showed several bouts of obstructive apnea and oxygen desaturation. He was referred for urgent adenotomy. At the age of 4 years and 5 months (3 months after surgery), the symptomatology and repeat sleep polygraph study showed a marked improvement. In conclusion, obstructive sleep apnea is a very hazardous condition for children with Noonan syndrome. They are a population at risk because of facial problems and cardiopathy. The real global incidence of obstructive sleep apnea syndrome in these children is unknown.https://www.jpnim.com/index.php/jpnim/article/view/631obstructive sleep apnea syndromenoonan syndromepolygraphysleep study
spellingShingle Marco Zaffanello
Antonia Maria Prioli
Luca Sacchetto
Emma Gasperi
Alfonso Bisceglia
Anna Ludovica Ghobert
Giorgio Piacentini
Sleep disordered breathing in Noonan syndrome: a rare case report
Journal of Pediatric and Neonatal Individualized Medicine
obstructive sleep apnea syndrome
noonan syndrome
polygraphy
sleep study
title Sleep disordered breathing in Noonan syndrome: a rare case report
title_full Sleep disordered breathing in Noonan syndrome: a rare case report
title_fullStr Sleep disordered breathing in Noonan syndrome: a rare case report
title_full_unstemmed Sleep disordered breathing in Noonan syndrome: a rare case report
title_short Sleep disordered breathing in Noonan syndrome: a rare case report
title_sort sleep disordered breathing in noonan syndrome a rare case report
topic obstructive sleep apnea syndrome
noonan syndrome
polygraphy
sleep study
url https://www.jpnim.com/index.php/jpnim/article/view/631
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AT emmagasperi sleepdisorderedbreathinginnoonansyndromeararecasereport
AT alfonsobisceglia sleepdisorderedbreathinginnoonansyndromeararecasereport
AT annaludovicaghobert sleepdisorderedbreathinginnoonansyndromeararecasereport
AT giorgiopiacentini sleepdisorderedbreathinginnoonansyndromeararecasereport