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Sleep disordered breathing and airway disease in primary ciliary dyskinesia

Francesca Santamaria1, Maria Esposito, Silvia Montella

  • 1Department 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.

Respirology (Carlton, Vic.)
|March 26, 2014
PubMed

Insights

Sleep-disordered breathing (SDB) is common in primary ciliary dyskinesia (PCD) and linked to lung issues. Early detection and management of SDB are crucial for improving PCD patient outcomes.

Area of Science:

  • Pediatric Pulmonology
  • Sleep Medicine
  • Genetics

Background:

  • Primary ciliary dyskinesia (PCD) is a genetic disorder affecting cilia function.
  • Sleep-disordered breathing (SDB) can exacerbate PCD, creating a negative feedback loop.
  • Understanding the relationship between PCD and SDB is vital for comprehensive patient care.

Purpose of the Study:

  • To investigate the prevalence and characteristics of SDB in children with PCD.
  • To explore the association between SDB, respiratory function, and structural lung changes in PCD patients.
  • To determine if SDB contributes to the pulmonary morbidity observed in PCD.

Main Methods:

  • Overnight respiratory polysomnography (rPSG) and the Sleep Disturbances Scale for Children (SDSC) were performed on 16 stable PCD patients and 42 controls.
  • PCD patients also underwent nasal endoscopy, pulmonary function tests, and chest high-resolution computed tomography (HRCT).
  • Statistical analyses were used to compare SDB parameters between groups and correlate them with clinical and imaging findings.

Main Results:

  • PCD patients exhibited significantly higher rates of obstructive and central apneas, hypopneas, and oxygen desaturation compared to controls.
  • No significant correlation was found between SDSC scores and rPSG findings in PCD patients.
  • SDB indicators, including oxygen desaturation index (ODI), correlated with lung function (functional residual capacity) and structural abnormalities (HRCT scores, peribronchial thickening, bronchiectasis severity).

Conclusions:

  • Sleep-disordered breathing is highly prevalent in children with primary ciliary dyskinesia.
  • Nocturnal desaturation in PCD is associated with impaired lung function and structural lung damage.
  • SDB may be an underrecognized contributor to the pulmonary morbidity in primary ciliary dyskinesia.
Abstract

Keywords:
lower airwayobstructive sleep apnoea syndromeprimary ciliary dyskinesiasleep-disordered breathingupper airway

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