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Obstructive Sleep Apnea in MPS: A Systematic Review of Pretreatment and Posttreatment Prevalence and Severity
Pal, Abhijit Ricky; Brown, Nailah; Jones, Simon A.; Bigger, Brian W.; Bruce, Iain A..
Afiliação
  • Pal, Abhijit Ricky; Manchester Academic Health Science Centre. Central Manchester University Hospitals NHS Foundation Trust. Royal Manchester Children's Hospital. Manchester. GB
  • Brown, Nailah; Manchester Academic Health Science Centre. Central Manchester University Hospitals NHS Foundation Trust. Royal Manchester Children's Hospital. Manchester. GB
  • Jones, Simon A.; St. Mary's Hospital. Manchester Centre for Genomic Medicine. Willink Biochemical Genetics Unit. Manchester. GB
  • Bigger, Brian W.; University of Manchester. Faculty of Medical and Human Sciences. Institute of Human Development. Manchester. GB
  • Bruce, Iain A.; Manchester Academic Health Science Centre. Central Manchester University Hospitals NHS Foundation Trust. Royal Manchester Children's Hospital. Manchester. GB
J. inborn errors metab. screen ; 3: e150008, 2015. tab, graf
Article em En | LILACS-Express | LILACS | ID: biblio-1090868
Biblioteca responsável: BR1.1
ABSTRACT
Abstract The mucopolysaccharidoses (MPSs) are a group of inherited, metabolic disorders characterized by progressive multisystem accumulation of partially degraded glycosaminoglycans. This manifests with multilevel airway obstruction, presenting with obstructive sleep apnea (OSA). We systematically reviewed the literature to determine the severity and prevalence of OSA in MPS based on polysomnography analysis. Fifteen studies with 294 participants met the inclusion criteria for review. The pretreatment prevalence of OSA in MPS was 81% with a mean apnea-hypopnea index (AHI) of 10.4. Patients with MPS I are most significantly affected, with 75% suffering with moderate to severe OSA (mean AHI, 16.6). Enzyme replacement therapy (ERT) results in an almost significant reduction in OSA in MPS I (P = .06), while adenotonsillar surgery significantly improves AHI (P = .002). Obstructive sleep apnea least affects MPS III. There is a lack of long-term post-ERT and hematopoietic stem cell transplant data relating to OSA outcomes in this population, with further prospective studies required to determine the ongoing response to treatment.
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Texto completo: 1 Coleções: 01-internacional Base de dados: LILACS Tipo de estudo: Observational_studies / Prevalence_studies / Risk_factors_studies / Systematic_reviews Idioma: En Revista: J. inborn errors metab. screen Assunto da revista: Medicina Cl¡nica / Patologia Ano de publicação: 2015 Tipo de documento: Article País de afiliação: Reino Unido País de publicação: Brasil
Texto completo
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Texto completo: 1 Coleções: 01-internacional Base de dados: LILACS Tipo de estudo: Observational_studies / Prevalence_studies / Risk_factors_studies / Systematic_reviews Idioma: En Revista: J. inborn errors metab. screen Assunto da revista: Medicina Cl¡nica / Patologia Ano de publicação: 2015 Tipo de documento: Article País de afiliação: Reino Unido País de publicação: Brasil