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

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Date
2014-12-01
Author
Moreira, Gustavo A.
Kyosen, Sandra O. [UNIFESP]
Patti, Camilla L. [UNIFESP]
Martins, Ana Maria [UNIFESP]
Tufik, Sergio [UNIFESP]
Type
Artigo
ISSN
1520-9512
Is part of
Sleep and Breathing
DOI
10.1007/s11325-014-0944-6
Metadata
Show full item record
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.
Citation
Sleep and Breathing. Heidelberg: Springer Heidelberg, v. 18, n. 4, p. 791-797, 2014.
Keywords
Lysosomal storage diseases
Inborn error of metabolism
Sleep-disordered breathing
Hypoxia
Polysomnography
Sponsorship
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
URI
http://repositorio.unifesp.br/handle/11600/38499
Collections
  • EPM - Artigos [17709]

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