Martens Marilee
Department of Psychology, Ohio State University, Newark, OH, USA.
Handb Clin Neurol. 2013;111:291-3. doi: 10.1016/B978-0-444-52891-9.00033-6.
Williams syndrome (WS) is a neurodevelopmental genetic disorder with a prevalence of approximately 1 in 7500. It is caused by a hemizygous deletion of approximately 28 genes on the 7th chromosome (7q11.23), including the elastin allele. Individuals with WS have atypical facial characteristics, including medial eyebrow flair, epicanthal folds, upturned nose, elongated philtrum, and full lips. Infants typically experience constipation and colic. Cardiac and renal abnormalities, as well as hypertension, are common. Individuals with WS are at increased risk for hearing and vision difficulties. Gastrointestinal difficulties are common and include constipation and diverticulitis. Joint abnormalities are observed in both children and adults with WS. Individuals with WS are overly friendly yet can experience nonsocial anxieties. Cognitive skills are typically in the mild to moderately delayed range. Relative strengths have been demonstrated in some aspects of language and face processing, while visuospatial skills are significantly delayed.
威廉姆斯综合征(WS)是一种神经发育性遗传疾病,患病率约为七千五百分之一。它由第7号染色体(7q11.23)上约28个基因的半合子缺失引起,包括弹性蛋白等位基因。患有WS的个体具有非典型面部特征,包括内侧眉梢上扬、内眦赘皮、鼻尖上翘、人中拉长和嘴唇丰满。婴儿通常会出现便秘和腹绞痛。心脏和肾脏异常以及高血压很常见。患有WS的个体听力和视力困难风险增加。胃肠道问题很常见,包括便秘和憩室炎。患有WS的儿童和成人都观察到关节异常。患有WS的个体过度友好,但可能会经历非社交焦虑。认知技能通常处于轻度至中度延迟范围。在语言和面部处理的某些方面已显示出相对优势,而视觉空间技能则明显延迟。