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学龄前原发性纤毛运动障碍患儿的发育和行为问题。

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

机构信息

Faculty of Medicine, Department of Pediatrics, Division of Developmental Pediatrics, Hacettepe University, Ankara, Turkey.

Faculty of Medicine, Department of Pediatrics, Department of Pediatric Pulmonology, Hacettepe University, Ankara, Turkey.

出版信息

Eur J Pediatr. 2019 Jul;178(7):995-1003. doi: 10.1007/s00431-019-03382-z. Epub 2019 Apr 27.

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.

摘要

原发性纤毛运动障碍(PCD)可引起广泛的疾病。本研究旨在探讨学龄前PCD 患儿的发育、行为和社会情感方面。纳入 14 名 PCD 患儿、17 名囊性纤维化(CF)患者和 15 名健康对照。采用年龄与阶段问卷评估参与者的发育特征。参与者的父母填写儿童行为检查表(CBCL)。PCD 组有更多的儿童筛查出发育迟缓。PCD 组在沟通和解决问题领域筛查出发育迟缓的儿童数量更多。与健康对照组相比,PCD 和 CF 患儿精细运动技能发育迟缓更为常见。三组在粗大运动和个人-社会发育方面无差异。三组儿童均无社会情感问题。在 CBCL 中,CF 患者的内化问题评分较高。三组之间的外化问题和总问题评分无差异。然而,在 PCD 患者中,有一个以上领域发育迟缓的儿童有更高的外化问题和总问题评分。结论:本研究显示,与 CF 患者和健康儿童相比,学龄前 PCD 患儿发育迟缓的阳性筛查更为常见。已知:• 尽管 PCD 患儿在儿童行为检查表(CBCL)上的内化问题评分较高,但他们的学龄期智力评分与健康对照相似。• 学龄期 PCD 患者表现出更高的多动和注意力不集中表现。新发现:• 学龄前 PCD 患儿在沟通、解决问题和精细运动技能方面存在发育迟缓的阳性筛查更为常见。• 在 CBCL 上筛查出有一个以上领域发育迟缓的学龄前 PCD 患儿有更高的外化问题和总问题评分。

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