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Evaluation of sleep-disordered breathing and its relationship with respiratory parameters in children with mucopolysaccharidosis Type IVA and VI.
Ademhan Tural, Dilber; Emiralioglu, Nagehan; Dogru, Deniz; Ozsezen, Beste; Ipek, Omer Faruk; Sunman, Birce; Nayir Buyuksahin, Halime; Guzelkas, Ismail; Ciki, Kismet; Kilic, Kubra; Vardar Yagli, Naciye; Yalcin, Ebru; Kiper, Nural; Sivri, Serap; Ozcelik, Ugur.
Afiliación
  • Ademhan Tural D; Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, Ihsan Dogramaci Children's Hospital, Ankara, Turkey.
  • Emiralioglu N; Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, Ihsan Dogramaci Children's Hospital, Ankara, Turkey.
  • Dogru D; Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, Ihsan Dogramaci Children's Hospital, Ankara, Turkey.
  • Ozsezen B; Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, Ihsan Dogramaci Children's Hospital, Ankara, Turkey.
  • Ipek OF; Department of Pediatrics, School of Medicine, Hacettepe University, Ihsan Dogramaci Children's Hospital, Ankara, Turkey.
  • Sunman B; Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, Ihsan Dogramaci Children's Hospital, Ankara, Turkey.
  • Nayir Buyuksahin H; Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, Ihsan Dogramaci Children's Hospital, Ankara, Turkey.
  • Guzelkas I; Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, Ihsan Dogramaci Children's Hospital, Ankara, Turkey.
  • Ciki K; Department of Pediatric Metabolism, Ihsan Dogramaci Children's Hospital, Hacettepe University Faculty of Medicine, Ankara, Turkey.
  • Kilic K; Department of Physical Therapy and Rehabilitation, Faculty of Medical Sciences, Hacettepe University, Ankara, Turkey.
  • Vardar Yagli N; Department of Physical Therapy and Rehabilitation, Faculty of Medical Sciences, Hacettepe University, Ankara, Turkey.
  • Yalcin E; Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, Ihsan Dogramaci Children's Hospital, Ankara, Turkey.
  • Kiper N; Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, Ihsan Dogramaci Children's Hospital, Ankara, Turkey.
  • Sivri S; Department of Pediatric Metabolism, Ihsan Dogramaci Children's Hospital, Hacettepe University Faculty of Medicine, Ankara, Turkey.
  • Ozcelik U; Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, Ihsan Dogramaci Children's Hospital, Ankara, Turkey.
Am J Med Genet A ; 185(8): 2306-2314, 2021 08.
Article en En | MEDLINE | ID: mdl-33960649
The aims of the study were to evaluate the prevalence of sleep-disordered breathing (SDB) by using polysomnography (PSG) in children with MPS IVA and MPS VI who underwent enzyme replacement therapy (ERT) and to analyze the effect on SDB of having upper airway surgery, pulmonary functions, and exercise capacity. A retrospective cross-sectional study was conducted on patients with MPS IVA (n:17) and MPS VI (n:11) aged under 19 years who underwent polysomnography. Descriptive and nonparametric analyses were performed for demographic, PSG, pulmonary function and exercise capacity variables. The frequency of sleep apnea in the study sample was 85.7% (24/28). Four patients (14.3%) had no sleep apnea, 15 (53.6%) had mild, and nine (32.1%) had moderate-to-severe sleep apnea. Two patients (7.1%) had central sleep apnea and 22 had obstructive sleep apnea (OSA) (78.6%). Forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC) were negatively correlated to apnea-hypopnea index (AHI) (r = -0.594, p = .009; r = -0.636, p = .005, respectively). Despite ERT and previous upper airway surgery, the prevalence of OSA was high in patients with MPS IVA-MPS IV, emphasizing the importance of PSG screening for sleep disorders. Pulmonary function tests may be useful for predicting sleep apnea in patients with MPS IVA and MPS VI.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Síndromes de la Apnea del Sueño / Mucopolisacaridosis IV Tipo de estudio: Diagnostic_studies / Etiology_studies / Observational_studies / Prevalence_studies / Prognostic_studies / Risk_factors_studies Límite: Adolescent / Child / Child, preschool / Female / Humans / Infant / Male / Newborn Idioma: En Revista: Am J Med Genet A Asunto de la revista: GENETICA MEDICA Año: 2021 Tipo del documento: Article País de afiliación: Turquía Pais de publicación: Estados Unidos
Texto completo
Añadir a Mi BVS
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Síndromes de la Apnea del Sueño / Mucopolisacaridosis IV Tipo de estudio: Diagnostic_studies / Etiology_studies / Observational_studies / Prevalence_studies / Prognostic_studies / Risk_factors_studies Límite: Adolescent / Child / Child, preschool / Female / Humans / Infant / Male / Newborn Idioma: En Revista: Am J Med Genet A Asunto de la revista: GENETICA MEDICA Año: 2021 Tipo del documento: Article País de afiliación: Turquía Pais de publicación: Estados Unidos