Williams综合征儿童早期认知发育特征：一项前瞻性队列研究

doi:10.7499/j.issn.1008-8830.2404029

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摘要目的调查不同年龄段Williams综合征（Williams syndrome, WS）儿童早期的认知发育特点。方法前瞻性纳入2018年9月—2023年6月期间在浙江大学医学院附属儿童医院儿童保健科门诊确诊为WS的儿童106例，年龄1~<5岁。所有患儿均进行了Gesell发育诊断评估。分析不同年龄段WS儿童的认知发育特征。结果 106例WS儿童的平均年龄为（3.1±1.2）岁；男性58例，女性48例。男女患儿在大运动、精细动作、语言、个人-社会及适应性行为这5个能区发育水平的比较差异均无统计学意义（P>0.05）。不同年龄段患儿轻度、中度、重度和极重度发育障碍的发生率差异均无统计学意义（P>0.05）。不同年龄段患儿大运动、语言、个人-社会、适应性等4个能区发育水平的比较差异无统计学意义（P>0.05）；随着年龄的增长，患儿的精细动作发育水平呈下降的趋势（P<0.05）。WS儿童中，每个年龄组的言语智商和非言语智商的比较差异均无统计学意义（P>0.05）。结论 WS儿童整体的发育水平随年龄增长保持稳定，其早期的语言能力并没有显著优于非语言能力。

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	姚丹
	李芳芳
	沈季阳
	陈维军
	季钗

关键词 ： Williams综合征, 认知发育, 言语, 儿童

Abstract：Objective To investigate the early cognitive development characteristics of children with Williams syndrome (WS) at different age stages. Methods From September 2018 to June 2023, 106 children diagnosed with WS at the Department of Pediatric Health Care, Children's Hospital, Zhejiang University School of Medicine, aged 1 to <5 years, were prospectively enrolled. All children underwent Gesell developmental diagnostic assessments to analyze the cognitive development characteristics of WS children across different age groups. Results The average age of the 106 WS children was (3.1±1.2) years; 58 were male, and 48 were female. There were no significant differences in developmental levels between males and females in the five domains of gross motor skills, fine motor skills, language, personal-social skills, and adaptive behavior (P>0.05). The incidence rates of mild, moderate, severe, and profound developmental disabilities among children of different age groups showed no significant differences (P>0.05). Comparisons of developmental levels in gross motor skills, language, personal-social skills, and adaptive behavior among different age groups were also not statistically significant (P>0.05). With the increase of age, the developmental level of fine motor skills showed a decreasing trend (P<0.05). There were no significant differences in verbal IQ and non-verbal IQ within each age group of WS children (P>0.05). Conclusions The overall developmental level of WS children stabilizes with age, and their early language abilities do not significantly exceed their non-verbal abilities.

Key words： Williams syndrome Cognitive development Language Child

收稿日期: 2024-04-03

通讯作者: 季钗，女，副主任医师。Email：6196021@zju.edu.cn。 E-mail: 6196021@zju.edu.cn

作者简介: 姚丹，女，硕士，副主任医师；

引用本文:

姚丹,李芳芳,沈季阳等. Williams综合征儿童早期认知发育特征：一项前瞻性队列研究[J]. 中国当代儿科杂志, 2024, 26(10): 1053-1057.

YAO Dan,LI Fang-Fang,SHEN Ji-Yang et al. Characteristics of early cognitive development in children with Williams syndrome: a prospective cohort study[J]. CJCP, 2024, 26(10): 1053-1057.

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