title: Sleep disordered breathing and airway disease in primary ciliary dyskinesia.
abstract: OBJECTIVE
  Sleep-disordered breathing (SDB) may develop in primary ciliary dyskinesia (PCD), leading to these diseases worsening one another.
  
  
  METHODS
  Sixteen stable PCD patients (4.9-17.2 years) and 42 controls underwent overnight respiratory polysomnography (rPSG) and Sleep Disturbances Scale for Children (SDSC). In PCD we assessed nasal endoscopy, pulmonary function tests and chest high-resolution computed tomography (HRCT).
  
  
  RESULTS
  Compared with controls, PCD had higher obstructive apnoea (4.7 vs 0.2, P < 0.001), central apnoea (0.8 vs 0.2, P < 0.001), hypopnoea (1.8 vs 0.2, P < 0.001), apnoea-hypopnoea (7.8 vs 0.6, P < 0.001), oxygen desaturation indexes (ODI; 0.7 vs 0.2, P = 0.002), and mean oxygen desaturation (4% vs 1%, P < 0.001), while mean and nadir oxygen saturation (97.1% vs 98.1, P < 0.001) (93% vs 97.2%, P < 0.001) were lower, respectively. In PCD, SDSC was unrelated to rPSG (P > 0.05), with total score and subscores of disorders in initiating and maintaining sleep, and sleep-wake transition lower than controls. PCD patients had chronic rhinosinusitis (100%) and adenoidal hypertrophy (50%). Total HRCT score was 7 (range 0-14). ODI correlated with functional residual capacity (r = 0.8, P = 0.02), total HRCT (r = 0.6, P = 0.03) and peribronchial thickening scores (r = 0.7, P = 0.02). Oxygen saturation was associated with bronchiectasis severity score (r = -0.6, P = 0.02).
  
  
  CONCLUSIONS
  PCD's parents may underestimate SDB. As nocturnal desaturation is associated with lung function and structure abnormalities, SDB may significantly contribute to pulmonary morbidity.
authors.affiliation: Department of Translational Medical Sciences, Reproductive and Odontostomatologic Sciences, Unit of Ear, Nose and Throat and Molecular and Cellular Biology and Pathology, Federico II University, Naples, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Santamaria
authors.forename: Maria
authors.initials: M
authors.lastname: Esposito
authors.forename: Silvia
authors.initials: S
authors.lastname: Montella
authors.forename: Elena
authors.initials: E
authors.lastname: Cantone
authors.forename: Carmine
authors.initials: C
authors.lastname: Mollica
authors.forename: Sara
authors.initials: S
authors.lastname: De Stefano
authors.forename: Virginia
authors.initials: V
authors.lastname: Mirra
authors.forename: Marco
authors.initials: M
authors.lastname: Carotenuto
mesh_terms: D000328:Adult; D002648:Child; D054626:Consumer Health Information; D003430:Cross-Sectional Studies; D005260:Female; D007722:Health Knowledge, Attitudes, Practice; D006801:Humans; D007558:Italy / Q000453:epidemiology; D007619:Kartagener Syndrome / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology; D008297:Male; D009035:Mothers / Q000193:education / Q000523:psychology; D017286:Polysomnography / Q000379:methods; D011446:Prospective Studies; D012129:Respiratory Function Tests / Q000379:methods; D018570:Risk Assessment; D012307:Risk Factors; D012720:Severity of Illness Index; D012891:Sleep Apnea Syndromes / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000503:physiopathology / Q000523:psychology; D014057:Tomography, X-Ray Computed / Q000379:methods
keywords: lower airway; obstructive sleep apnoea syndrome; primary ciliary dyskinesia; sleep-disordered breathing; upper airway