Selås Magnhild, Helland Wenche Andersen
Department of Biological and Medical Psychology, Faculty of Psychology, University of Bergen, Bergen, Norway.
Section of Research & Innovation, Helse Fonna, Haugesund, Norway.
Clin Linguist Phon. 2016;30(11):899-910. doi: 10.1080/02699206.2016.1188422. Epub 2016 Jun 27.
Noonan syndrome (NS) is a disorder causing symptoms like short stature, characteristic facial features, congenital heart disease, possible mental retardation, and pragmatic difficulties. This study describes the pragmatic skills in NS and discusses the linguistic profile of 17 informants aged 6-15 years, by comparing the participants' scores on the Children's Communication Checklist, 2nd edition (CCC-2) (Bishop, 2011), with a group of typically developing children of matching age and gender. Language impairments were common in the NS group. The results show that children and adolescents with NS do not have one coherent pragmatic profile. However, 76.5% of the participants displayed communication impairments, and pragmatic skills were significantly lower than in the control group.
努南综合征（NS）是一种导致身材矮小、特征性面部特征、先天性心脏病、可能的智力迟钝和语用困难等症状的疾病。本研究描述了努南综合征患者的语用技能，并通过比较17名6至15岁受试者在《儿童沟通检查表》第二版（CCC-2）（毕晓普，2011年）上的得分与一组年龄和性别匹配的发育正常儿童，探讨了他们的语言特征。语言障碍在努南综合征组中很常见。结果表明，患有努南综合征的儿童和青少年没有一个连贯的语用特征。然而，76.5%的受试者表现出沟通障碍，其语用技能明显低于对照组。
