title: Prader Willi syndrome and obstructive sleep apnea: co-occurrence in the pediatric population.
abstract: BACKGROUND
  A high prevalence of obstructive sleep apnea (OSA) occurs in children with Prader-Willi syndrome (PWS). Yet, due in part to the relatively small samples previously used, the prevalence of OSA has varied greatly across studies. It is also unclear if factors such as age, gender, body mass index (BMI), or type of genetic imprinting are associated with increased risk for OSA among children with PWS.
  
  
  OBJECTIVE
  To evaluate the (a) prevalence of OSA, as well as narcolepsy, in pediatric populations diagnosed with PWS; (b) effects of age, gender, body mass index, and genetic imprinting on OSA severity; and (c) efficacy of adenotonsillectomy (AT) for decreasing OSA severity in this population.
  
  
  METHODS
  All studies assessing OSA among children with PWS through August 2013 were identified using the PubMed/Medline, Psych Info, Cochrane library, and Google Scholar data bases.
  
  
  RESULTS
  Fourteen studies of children diagnosed with PWS and who were assessed for OSA using polysomnography (PSG) met inclusion criteria (n = 224 children). The prevalence of OSA across studies was 79.91% (n = 179/224). Among youths with OSA, 53.07% had mild OSA, 22.35% moderate OSA, and 24.58% severe OSA. Narcolepsy was found to occur in 35.71% of children with PWS. Adenotonsillectomy was associated with improvement in OSA for most children with PWS. However, residual OSA was present in the majority of cases post-surgery.
  
  
  CONCLUSIONS
  This study confirms the high prevalence of OSA and narcolepsy among children with PWS. Screening for OSA and narcolepsy among children with PWS is recommended. In addition, while adenotonsillectomy was effective in reducing OSA for some children, alternative treatments may need to be considered, given the only moderate response rate.
authors.affiliation: Department of Psychiatry, Cooper University Hospital, Camden, NJ.
authors.forename: Karim
authors.initials: K
authors.lastname: Sedky
authors.affiliation: Department of Psychiatry, Drexel University College of Medicine, Philadelphia, PA.
authors.forename: David S
authors.initials: DS
authors.lastname: Bennett
authors.affiliation: Department of Psychiatry, Cooper University Hospital, Camden, NJ.
authors.forename: Andres
authors.initials: A
authors.lastname: Pumariega
mesh_terms: D000233:Adenoidectomy; D000293:Adolescent; D000367:Age Factors; D015992:Body Mass Index; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009290:Narcolepsy / Q000150:complications / Q000453:epidemiology; D011218:Prader-Willi Syndrome / Q000150:complications / Q000453:epidemiology*; D015995:Prevalence; D012720:Severity of Illness Index; D012737:Sex Factors; D020181:Sleep Apnea, Obstructive / Q000150:complications / Q000453:epidemiology* / Q000601:surgery; D014068:Tonsillectomy; D016896:Treatment Outcome; D055815:Young Adult
keywords: Prader-Willi syndrome; meta-analysis; narcolepsy; obstructive sleep apnea; sleep disordered breathing
references.citation: Eur J Neurol. 2005 Jan;12(1):70-2
references.pmid: 15613151
references.citation: Clin Neurophysiol. 2005 Nov;116(11):2685-92
references.pmid: 16213786
references.citation: Am J Med Genet A. 2011 Jul;155A(7):1552-7
references.pmid: 21671379
references.citation: Proc Am Thorac Soc. 2008 Feb 15;5(2):274-82
references.pmid: 18250221
references.citation: J Clin Endocrinol Metab. 2003 May;88(5):2206-12
references.pmid: 12727976
references.citation: J Paediatr Child Health. 2013 Mar;49(3):238-41
references.pmid: 23438058
references.citation: Chest. 2004 Mar;125(3):872-8
references.pmid: 15006944
references.citation: Arch Otolaryngol Head Neck Surg. 2012 Nov;138(11):1047-51
references.pmid: 23165379
references.citation: Neuropsychol Rev. 2006 Sep;16(3):123-9
references.pmid: 17109239
references.citation: Am J Med Genet. 2000 Summer;97(2):136-46
references.pmid: 11180221
references.citation: Chest. 1995 Dec;108(6):1627-31
references.pmid: 7497773
references.citation: J Obes. 2012;2012:473941
references.pmid: 23133744
references.citation: Pediatr Pulmonol. 2006 Jan;41(1):74-9
references.pmid: 16283627
references.citation: J Clin Sleep Med. 2008 Apr 15;4(2):111-8
references.pmid: 18468308
references.citation: Chest. 2001 Dec;120(6):1930-5
references.pmid: 11742924
references.citation: Am J Respir Crit Care Med. 1999 May;159(5 Pt 1):1527-32
references.pmid: 10228121
references.citation: Obes Res. 2005 Jul;13(7):1175-9
references.pmid: 16076986
references.citation: Pediatr Pulmonol. 2002 Sep;34(3):209-17
references.pmid: 12203850
references.citation: J Clin Endocrinol Metab. 2006 Dec;91(12):4911-5
references.pmid: 17003096
references.citation: Arch Pediatr Adolesc Med. 1996 Dec;150(12):1288-93
references.pmid: 8954002
references.citation: Ann Otol Rhinol Laryngol. 2009 Apr;118(4):267-9
references.pmid: 19462846
references.citation: J Pediatr. 2013 Feb;162(2):263-8.e1
references.pmid: 22947651
references.citation: Proc Am Thorac Soc. 2008 Feb 15;5(2):136-43
references.pmid: 18250205
references.citation: Pediatr Pulmonol. 2007 Oct;42(10):881-7
references.pmid: 17722117
references.citation: Sleep. 2010 Nov;33(11):1457-64
references.pmid: 21102987
references.citation: J Clin Endocrinol Metab. 2006 Feb;91(2):413-7
references.pmid: 16317059
references.citation: Respir Physiol Neurobiol. 2009 Aug 31;168(1-2):119-24
references.pmid: 19712904
references.citation: J Pediatr. 2005 Dec;147(6):823-9
references.pmid: 16356439
references.citation: N Engl J Med. 2005 Nov 10;353(19):2034-41
references.pmid: 16282178
references.citation: Int J Pediatr Otorhinolaryngol. 2010 Nov;74(11):1270-2
references.pmid: 20880597
references.citation: Clin Neurophysiol. 2001 May;112(5):800-5
references.pmid: 11336895
references.citation: Arch Pediatr Adolesc Med. 1999 Jan;153(1):33-7
references.pmid: 9894997
references.citation: J Clin Sleep Med. 2013 Nov 15;9(11):1213-20
references.pmid: 24235907
references.citation: Chest. 2001 Jul;120(1):151-5
references.pmid: 11451831
references.citation: Otolaryngol Head Neck Surg. 2009 Jun;140(6):800-8
references.pmid: 19467393
references.citation: Pediatrics. 1993 Feb;91(2):398-402
references.pmid: 8424017
references.citation: Psychiatry Clin Neurosci. 2000 Jun;54(3):323-5
references.pmid: 11186096
references.citation: Sleep Med Rev. 2014 Aug;18(4):349-56
references.pmid: 24581717
references.citation: Pediatrics. 2007 Oct;120(4):e1028-34
references.pmid: 17908723
references.citation: Proc Am Thorac Soc. 2008 Feb 15;5(2):193-9
references.pmid: 18250212
references.citation: Pediatrics. 2002 Apr;109(4):e69
references.pmid: 11927742
references.citation: Sleep Breath. 2010 Jun;14(2):167-70
references.pmid: 19763652
references.citation: Paediatr Respir Rev. 2002 Mar;3(1):25-35
references.pmid: 12065179
references.citation: Am J Med Genet A. 2008 Dec 1;146A(23):3018-25
references.pmid: 19006209