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Published on: January 19, 2022
Developmental and behavioral problems in preschool-aged primary ciliary dyskinesia patients
P Zengin Akkus1, M Gharibzadeh Hizal2, E Ilter Bahadur3
1Faculty of Medicine, Department of Pediatrics, Division of Developmental Pediatrics, Hacettepe University, Ankara, Turkey. pinarzenginakkus@gmail.com.
Insights
Preschoolers with primary ciliary dyskinesia (PCD) show higher rates of developmental delays, particularly in communication and problem-solving. These delays are linked to increased behavioral issues in children with PCD.
Area of Science:
- Pediatric Health
- Developmental Pediatrics
- Rare Diseases
Background:
- Primary ciliary dyskinesia (PCD) is a rare genetic disorder affecting cilia function, leading to a range of health issues.
- While intelligence is comparable to healthy peers, school-aged PCD patients exhibit higher hyperactivity and inattention.
- The developmental, behavioral, and social-emotional profiles of preschool-aged children with PCD require further investigation.
Purpose of the Study:
- To explore developmental, behavioral, and social-emotional aspects in preschool-aged children diagnosed with PCD.
- To compare these aspects between children with PCD, cystic fibrosis (CF), and healthy controls.
Main Methods:
- Utilized the Ages and Stages Questionnaire for developmental screening.
- Employed the Child Behavior Checklist (CBCL) for behavioral and social-emotional assessment.
- Enrolled 14 PCD patients, 17 CF patients, and 15 healthy children.
Main Results:
- Children with PCD demonstrated a statistically higher incidence of positive developmental delay screenings compared to CF patients and healthy controls.
- Specific delays were more prevalent in communication and problem-solving domains for PCD patients.
- Fine motor skill delays were observed more frequently in both PCD and CF groups than in healthy children.
- No significant differences were found in gross motor or personal-social development across groups.
- No social-emotional problems were identified in any group.
- CF patients exhibited higher internalizing problem scores on the CBCL.
- PCD patients with delays in multiple domains showed elevated externalizing and total problem scores on the CBCL.
Conclusions:
- Preschool-aged children with PCD are more likely to screen positive for developmental delays than their peers with CF or healthy children.
- Developmental delays in PCD are associated with increased externalizing and total behavioral problems, particularly when delays span multiple domains.
Abstract:
Primary ciliary dyskinesia (PCD) causes a broad spectrum of disease. This study aims to explore the developmental, behavioral, and social-emotional aspects of preschool-aged children with PCD. Fourteen PCD, 17 cystic fibrosis (CF) patients and 15 healthy subjects were enrolled. Developmental features of the participants were evaluated with Ages and Stages Questionnaire. Parents of participants filled out the Child Behavior Checklist (CBCL). The number of children screened positive for developmental delay was statistically higher in the PCD group. Higher numbers of children with PCD were screened positive for developmental delay in communication and problem-solving domains. Delay in fine motor skill domain was more common in children with PCD and CF compared to healthy subjects. There was no difference among the three groups in terms of gross motor and personal-social development. None of the children in all three groups was shown to have social-emotional problems. In CBCL, patients with CF had higher internalizing problem scores. Externalizing and total problem scores did not differ between the three groups. However, among PCD patients, children with developmental delay on more than one domain had higher externalizing and total problem scores.Conclusion: The current study revealed that positive screening for developmental delay is more common in preschool-aged PCD patients compared to patients with CF and healthy children. What is Known: • Intelligence scores of school-aged PCD patients are similar to healthy subjects despite their higher internalizing problem scores on Child Behavior Checklist (CBCL). • School-aged PCD patients exhibit higher hyperactivity and inattention findings. What is New: • Positive screening for developmental delay in communication, problem-solving and fine motor skills is more common in preschool-aged PCD patients. • Preschool-aged PCD patients screened positive for developmental delay in more than one domain have higher externalizing and total problem scores on CBCL.
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