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Neuropsychological functioning of children with cystic fibrosis and primary ciliary dyskinesia. A systematic review.
  • Klára Benešová,
  • Eva Fürstová,
  • Tomáš Nikolai
Klára Benešová
Vseobecna fakultni nemocnice v Praze

Corresponding Author:[email protected]

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Eva Fürstová
Fakultni nemocnice v Motole Pediatricka klinika 2
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Tomáš Nikolai
Vseobecna fakultni nemocnice v Praze
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Cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) are chronic pulmonary conditions. In this review we focus on neuropsychological aspects of CF and PCD in children. Since studies conducted on children with asthma, sleep disturbed breathing (SBD), and studies on animal models describe specific cognitive impairments and suggest that intermittent hypoxia might be the pathophysiological mechanism. Main question of this systematic review is following: “Is neuropsychological functioning of children with CF and PCD specific?”. The database MedlineComplete was searched during September 2021 for each condition using specified key word combinations. We included studies in English, dealing only with pediatric population and papers published in academic journals within the last 10 years (2011-2021). We excluded case reports and studies using non-valid psychological methods or focusing on specific socio-economic populations. Applying these criteria, we acquired 5 studies in CF and 3 studies in PCD, out of them one study examined the association between cognitive impairment and oxygen desaturation levels. Five studies were scored high, and three studies were scored moderate level of evidence according to PRISMA and COCHRANE quality criteria. It can be concluded that the overall IQ of CF and PCD pediatric patients remains intact. However, impairments in specific cognitive functions, e.g., attention, memory or executive function have been described. Number of studies exploring neuropsychological functioning, especially in association with somatic aspects, in CF and PCD patients is not robust and further investigation is needed.