European Journal of Pediatrics

, Volume 178, Issue 7, pp 995–1003 | Cite as

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

  • P. Zengin AkkusEmail author
  • M. Gharibzadeh Hizal
  • E. Ilter Bahadur
  • E.N. Ozmert
  • S. Eryilmaz Polat
  • G. Ozdemir
  • S. Karahan
  • E. Yalcin
  • D. Dogru Ersoz
  • N. Kiper
  • U. Ozcelik
Original Article


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.


Primary ciliary dyskinesia Developmental delay Behavioral problems Social-emotional problems 



American Academy of Pediatrics


Ages and Stages Questionnaire


Ages and Stages Questionnaire:social-emotional


Ages and Stages Questionnaire for Turkish children


Child Behavior Checklist


Cystic fibrosis


Pediatric Sleep Questionnaire


Primary ciliary dyskinesia


Socio-economic status


Turkish version of the Ages and Stages Questionnaire:social-emotional


Authors’ contributions

Zengin Akkus P and Gharibzadeh Hizal M conceptualized and designed the study. They were responsible for all the assessments of patients. They drafted the initial manuscript, revised the manuscript, and approved the final manuscript.

Ilter Bahadur E, Esref S, and Ozdemir G were responsible for collecting data, they revised the manuscript and approved the final manuscript.

Karahan S carried out the statistical analyses, interpreted the statistical data, reviewed the manuscript, and approved the final manuscript.

Yalcin E, Dogru Ersoz D, and Kiper N interpreted the data, reviewed the manuscript, and approved the final manuscript.

Ozmert EN and Ozcelik U conceptualized and designed the study, supervised all the assessments of patients. They interpreted the statistical data, reviewed the manuscript, and approved the final manuscript.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Informed consent

Informed consent was obtained from parents of all participants included in the study.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. The Ethics Committee of the Hacettepe University Faculty of Medicine approved the study design. This article does not contain any studies with animals performed by any of the authors.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • P. Zengin Akkus
    • 1
    Email author
  • M. Gharibzadeh Hizal
    • 2
  • E. Ilter Bahadur
    • 1
  • E.N. Ozmert
    • 1
  • S. Eryilmaz Polat
    • 2
  • G. Ozdemir
    • 1
  • S. Karahan
    • 3
  • E. Yalcin
    • 2
  • D. Dogru Ersoz
    • 2
  • N. Kiper
    • 2
  • U. Ozcelik
    • 2
  1. 1.Faculty of Medicine, Department of Pediatrics, Division of Developmental PediatricsHacettepe UniversityAnkaraTurkey
  2. 2.Faculty of Medicine, Department of Pediatrics, Department of Pediatric PulmonologyHacettepe UniversityAnkaraTurkey
  3. 3.Faculty of Medicine, Department of BiostatisticsHacettepe UniversityAnkaraTurkey

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