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

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

Autor Moreira, Gustavo A. Google Scholar
Kyosen, Sandra O. Autor UNIFESP Google Scholar
Patti, Camilla L. Autor UNIFESP Google Scholar
Martins, Ana Maria Autor UNIFESP Google Scholar
Tufik, Sergio Autor UNIFESP Google Scholar
Instituição Inst Sono
Universidade Federal de São Paulo (UNIFESP)
Resumo 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.
Palavra-chave Lysosomal storage diseases
Inborn error of metabolism
Sleep-disordered breathing
Hypoxia
Polysomnography
Idioma Inglês
Financiador Instituto de Genetica e Erros Inatos do Metabolismo (IGEIM)
Biomarin
Shire
Associacao Fundo de Incentivo a Pesquisa (AFIP)
Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)
IGEIM
Data de publicação 2014-12-01
Publicado em Sleep and Breathing. Heidelberg: Springer Heidelberg, v. 18, n. 4, p. 791-797, 2014.
ISSN 1520-9512 (Sherpa/Romeo, fator de impacto)
Publicador Springer
Extensão 791-797
Fonte http://dx.doi.org/10.1007/s11325-014-0944-6
Direito de acesso Acesso restrito
Tipo Artigo
Web of Science WOS:000344784800017
Endereço permanente http://repositorio.unifesp.br/handle/11600/38499

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