Prevalence of obstructive sleep apnea in patients with mucopolysaccharidosis types I, II, and VI in a reference center

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dc.contributor.author Moreira, Gustavo A.
dc.contributor.author Kyosen, Sandra O. [UNIFESP]
dc.contributor.author Patti, Camilla L. [UNIFESP]
dc.contributor.author Martins, Ana Maria [UNIFESP]
dc.contributor.author Tufik, Sergio [UNIFESP]
dc.date.accessioned 2016-01-24T14:38:14Z
dc.date.available 2016-01-24T14:38:14Z
dc.date.issued 2014-12-01
dc.identifier http://dx.doi.org/10.1007/s11325-014-0944-6
dc.identifier.citation Sleep and Breathing. Heidelberg: Springer Heidelberg, v. 18, n. 4, p. 791-797, 2014.
dc.identifier.issn 1520-9512
dc.identifier.uri http://repositorio.unifesp.br/handle/11600/38499
dc.description.abstract Mucopolysaccharidosis (MPS) encompasses a group of rare lysosomal storage disorders that are associated with the accumulation of glycosaminoglycans in organs and tissues. Respiratory disorders occur in all MPS types. in these patients, the prevalence of obstructive sleep apnea syndrome (OSAS), which may confer additional morbidity, remains overlooked, and the results of the few existing studies are controversial. the present study aimed to characterize the prevalence of OSAS in patients with MPS types I, II, and VI in a reference center.Forty-five patients with MPS (I, n = 17; II, n = 16; and VI; n = 12) in the Centro de Referncia em Erros Inatos do Metabolismo, who underwent full-night polysomnography, were enrolled in a retrospective study. Demographic data and clinical history were collected from medical records of the first medical consultation.The prevalence of OSAS in patients with MPS was 69.8 %. MPS type I patients seemed to be more susceptible to OSA-induced hypoxemia, as indicated by reduced mean SpO(2) levels during both NREM and rapid eye movement sleep as well as during SpO(2) nadir.Patients with MPS displayed a high prevalence of OSAS, often with moderate to high severity. Together, our results reinforce the need for OSAS screening in all patients with MPS. en
dc.description.sponsorship Instituto de Genetica e Erros Inatos do Metabolismo (IGEIM)
dc.description.sponsorship Biomarin
dc.description.sponsorship Shire
dc.description.sponsorship Associacao Fundo de Incentivo a Pesquisa (AFIP)
dc.description.sponsorship Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)
dc.description.sponsorship IGEIM
dc.format.extent 791-797
dc.language.iso eng
dc.publisher Springer
dc.relation.ispartof Sleep and Breathing
dc.rights Acesso restrito
dc.subject Lysosomal storage diseases en
dc.subject Inborn error of metabolism en
dc.subject Sleep-disordered breathing en
dc.subject Hypoxia en
dc.subject Polysomnography en
dc.title Prevalence of obstructive sleep apnea in patients with mucopolysaccharidosis types I, II, and VI in a reference center en
dc.type Artigo
dc.rights.license http://www.springer.com/open+access/authors+rights?SGWID=0-176704-12-683201-0
dc.contributor.institution Inst Sono
dc.contributor.institution Universidade Federal de São Paulo (UNIFESP)
dc.description.affiliation Inst Sono, BR-04020060 São Paulo, Brazil
dc.description.affiliation Universidade Federal de São Paulo, Ctr Erros Inatos Metab CREIM, BR-04020041 São Paulo, Brazil
dc.description.affiliation Universidade Federal de São Paulo, Dept Psicobiol, São Paulo, Brazil
dc.description.affiliationUnifesp Universidade Federal de São Paulo, Ctr Erros Inatos Metab CREIM, BR-04020041 São Paulo, Brazil
dc.description.affiliationUnifesp Universidade Federal de São Paulo, Dept Psicobiol, São Paulo, Brazil
dc.identifier.doi 10.1007/s11325-014-0944-6
dc.description.source Web of Science
dc.identifier.wos WOS:000344784800017



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