Yao Dan, Li Fang-Fang, Shen Ji-Yang, Chen Wei-Jun, Ji Chai
Department of Pediatric Health Care, Children's Hospital, Zhejiang University School of Medicine/National Research Center for Child Health, Hangzhou 310003, China.
Zhongguo Dang Dai Er Ke Za Zhi. 2024 Oct 15;26(10):1053-1057. doi: 10.7499/j.issn.1008-8830.2404029.
To investigate the early cognitive development characteristics of children with Williams syndrome (WS) at different age stages.
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.
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 (>0.05). The incidence rates of mild, moderate, severe, and profound developmental disabilities among children of different age groups showed no significant differences (>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 (>0.05). With the increase of age, the developmental level of fine motor skills showed a decreasing trend (<0.05). There were no significant differences in verbal IQ and non-verbal IQ within each age group of WS children (>0.05).
The overall developmental level of WS children stabilizes with age, and their early language abilities do not significantly exceed their non-verbal abilities.
探讨威廉姆斯综合征（WS）患儿在不同年龄阶段的早期认知发展特征。
2018年9月至2023年6月，前瞻性纳入浙江大学医学院附属儿童医院儿童保健科诊断为WS的106例1至<5岁儿童。所有儿童均接受盖塞尔发育诊断评估，以分析不同年龄组WS儿童的认知发展特征。
106例WS儿童的平均年龄为（3.1±1.2）岁；男58例，女48例。粗大运动技能、精细运动技能、语言、个人社交技能和适应行为这五个领域中，男性和女性的发育水平无显著差异（>0.05）。不同年龄组儿童中轻度、中度、重度和极重度发育障碍的发生率无显著差异（>0.05）。不同年龄组在粗大运动技能、语言、个人社交技能和适应行为方面的发育水平比较也无统计学意义（>0.05）。随着年龄增长，精细运动技能的发育水平呈下降趋势（<0.05）。WS儿童各年龄组内的言语智商和非言语智商无显著差异（>0.05）。
WS儿童的整体发育水平随年龄增长而稳定，其早期语言能力并未显著超过非语言能力。
