Obstructive Sleep Apnea in MPS: A Systematic Review of Pretreatment and Posttreatment Prevalence and Severity
J. inborn errors metab. screen
;
3: e150008, 2015. tab, graf
Artigo
em Inglês
|
LILACS-Express
| LILACS
| ID: biblio-1090868
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.
Texto completo:
DisponíveL
Índice:
LILACS (Américas)
Tipo de estudo:
Estudo observacional
/
Estudo de prevalência
/
Fatores de risco
/
Revisões Sistemáticas Avaliadas
Idioma:
Inglês
Revista:
J. inborn errors metab. screen
Assunto da revista:
Medicina Cl¡nica
/
Patologia
Ano de publicação:
2015
Tipo de documento:
Artigo
País de afiliação:
Reino Unido
Instituição/País de afiliação:
Manchester Academic Health Science Centre/GB
/
St. Mary's Hospital/GB
/
University of Manchester/GB
Similares
MEDLINE
...
LILACS
LIS