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dc.contributor.authorMoreira, Gustavo A.
dc.contributor.authorKyosen, Sandra O. [UNIFESP]
dc.contributor.authorPatti, Camilla L. [UNIFESP]
dc.contributor.authorMartins, Ana Maria [UNIFESP]
dc.contributor.authorTufik, Sergio [UNIFESP]
dc.date.accessioned2016-01-24T14:38:14Z
dc.date.available2016-01-24T14:38:14Z
dc.date.issued2014-12-01
dc.identifierhttp://dx.doi.org/10.1007/s11325-014-0944-6
dc.identifier.citationSleep and Breathing. Heidelberg: Springer Heidelberg, v. 18, n. 4, p. 791-797, 2014.
dc.identifier.issn1520-9512
dc.identifier.urihttp://repositorio.unifesp.br/handle/11600/38499
dc.description.abstractMucopolysaccharidosis (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.sponsorshipInstituto de Genetica e Erros Inatos do Metabolismo (IGEIM)
dc.description.sponsorshipBiomarin
dc.description.sponsorshipShire
dc.description.sponsorshipAssociacao Fundo de Incentivo a Pesquisa (AFIP)
dc.description.sponsorshipConselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)
dc.description.sponsorshipIGEIM
dc.format.extent791-797
dc.language.isoeng
dc.publisherSpringer
dc.relation.ispartofSleep and Breathing
dc.rightsAcesso restrito
dc.subjectLysosomal storage diseasesen
dc.subjectInborn error of metabolismen
dc.subjectSleep-disordered breathingen
dc.subjectHypoxiaen
dc.subjectPolysomnographyen
dc.titlePrevalence of obstructive sleep apnea in patients with mucopolysaccharidosis types I, II, and VI in a reference centeren
dc.typeArtigo
dc.rights.licensehttp://www.springer.com/open+access/authors+rights?SGWID=0-176704-12-683201-0
dc.contributor.institutionInst Sono
dc.contributor.institutionUniversidade Federal de São Paulo (UNIFESP)
dc.description.affiliationInst Sono, BR-04020060 São Paulo, Brazil
dc.description.affiliationUniversidade Federal de São Paulo, Ctr Erros Inatos Metab CREIM, BR-04020041 São Paulo, Brazil
dc.description.affiliationUniversidade Federal de São Paulo, Dept Psicobiol, São Paulo, Brazil
dc.description.affiliationUnifespUniversidade Federal de São Paulo, Ctr Erros Inatos Metab CREIM, BR-04020041 São Paulo, Brazil
dc.description.affiliationUnifespUniversidade Federal de São Paulo, Dept Psicobiol, São Paulo, Brazil
dc.identifier.doi10.1007/s11325-014-0944-6
dc.description.sourceWeb of Science
dc.identifier.wosWOS:000344784800017


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