Developmental and behavioral problems in preschool-aged primary ciliary dyskinesia patients

ZENGİN AKKUŞ P., Gharibzadeh Hizal M., İLTER BAHADUR E., ÖZMERT E. N., Eryilmaz Polat S., ÖZDEMİR G., ...More

EUROPEAN JOURNAL OF PEDIATRICS, vol.178, no.7, pp.995-1003, 2019 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 178 Issue: 7
  • Publication Date: 2019
  • Doi Number: 10.1007/s00431-019-03382-z
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Social Sciences Citation Index (SSCI), Scopus
  • Page Numbers: pp.995-1003
  • Keywords: Primary ciliary dyskinesia, Developmental delay, Behavioral problems, Social-emotional problems, PEDIATRIC SLEEP QUESTIONNAIRE, CYSTIC-FIBROSIS, TURKISH VERSION, CHILDREN, DISEASE, RELIABILITY, PREVALENCE, VALIDITY, GENETICS
  • Hacettepe University Affiliated: Yes


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.