Background and objective. Sleep-disordered breathing (SDB) may develop in children with primary ciliary dyskinesia (PCD), leading to these diseases worsening one another. Assessment of SDB depends on detection of sleep disruption and quality parental report. Methods. Sixteen stable PCD patients (4.9-17.2 years) and forty-two controls underwent overnight respiratory polysomnography (rPSG) and Sleep Disturbances Scale for Children (SDSC). In PCD we assessed nasal obstruction by nasal endoscopy, and lung disease by pulmonary function tests and chest high resolution computed tomography (HRCT). Results. Compared to controls, PCD had higher obstructive apnoea (4.7 versus 0.2, p<0.001), central apnoea (0.8 versus 0.2, p<0.001), hypopnoea (1.8 versus 0.2, p<0.001), apnoea-hypopnoea (7.8 versus 0.6, p<0.001), oxygen desaturation indexes (ODI; 0.7 versus 0.2, p=0.002), and mean oxygen desaturation (4% versus 1%, p<0.001), while mean oxygen saturation (97.1% versus 98.1, p<0.001) and nadir oxygen saturation (93% versus 97.2%, p<0.001) were lower. In PCD, SDSC was unrelated to PSG (p> 0.05), with total score and subscores of disorders in initiating and maintaining sleep, and sleep-wake transition lower than controls. Nasal endoscopy revealed chronic rhinosinusitis and adenoidal hypertrophy in 100% and 50% of PCD, respectively. Total HRCT score was 7 (range, 0-14). ODI was related to 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). Mean oxygen saturation was associated with bronchiectasis severity score (r=-0.6, p=0.02). Conclusions. Parents of children with PCD may underestimate SDB. As nocturnal desaturation is associated to PCD lung function and structure abnormalities, SDB may significantly contribute to pulmonary morbidity.

Sleep disordered breathing and airway disease in primary ciliary dyskinesia

ESPOSITO, Maria;CAROTENUTO, Marco
2014

Abstract

Background and objective. Sleep-disordered breathing (SDB) may develop in children with primary ciliary dyskinesia (PCD), leading to these diseases worsening one another. Assessment of SDB depends on detection of sleep disruption and quality parental report. Methods. Sixteen stable PCD patients (4.9-17.2 years) and forty-two controls underwent overnight respiratory polysomnography (rPSG) and Sleep Disturbances Scale for Children (SDSC). In PCD we assessed nasal obstruction by nasal endoscopy, and lung disease by pulmonary function tests and chest high resolution computed tomography (HRCT). Results. Compared to controls, PCD had higher obstructive apnoea (4.7 versus 0.2, p<0.001), central apnoea (0.8 versus 0.2, p<0.001), hypopnoea (1.8 versus 0.2, p<0.001), apnoea-hypopnoea (7.8 versus 0.6, p<0.001), oxygen desaturation indexes (ODI; 0.7 versus 0.2, p=0.002), and mean oxygen desaturation (4% versus 1%, p<0.001), while mean oxygen saturation (97.1% versus 98.1, p<0.001) and nadir oxygen saturation (93% versus 97.2%, p<0.001) were lower. In PCD, SDSC was unrelated to PSG (p> 0.05), with total score and subscores of disorders in initiating and maintaining sleep, and sleep-wake transition lower than controls. Nasal endoscopy revealed chronic rhinosinusitis and adenoidal hypertrophy in 100% and 50% of PCD, respectively. Total HRCT score was 7 (range, 0-14). ODI was related to 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). Mean oxygen saturation was associated with bronchiectasis severity score (r=-0.6, p=0.02). Conclusions. Parents of children with PCD may underestimate SDB. As nocturnal desaturation is associated to PCD lung function and structure abnormalities, SDB may significantly contribute to pulmonary morbidity.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11591/199915
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