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Respirology. 2014 May;19(4):570-5. doi: 10.1111/resp.12273. Epub 2014 Mar 24.

Sleep disordered breathing and airway disease in primary ciliary dyskinesia.

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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.



Sleep-disordered breathing (SDB) may develop in primary ciliary dyskinesia (PCD), leading to these diseases worsening one another.


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).


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).


PCD's parents may underestimate SDB. As nocturnal desaturation is associated with lung function and structure abnormalities, SDB may significantly contribute to pulmonary morbidity.


lower airway; obstructive sleep apnoea syndrome; primary ciliary dyskinesia; sleep-disordered breathing; upper airway

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