Neuropsychological functioning of children with cystic fibrosis and
primary ciliary dyskinesia. A systematic review.
Abstract
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.