Hartshorne Nancy, Hudson Alexandra, MacCuspie Jillian, Kennert Benjamin, Nacarato Tasha, Hartshorne Timothy, Blake Kim
Social Sciences Division, Delta College, University Center, Michigan.
Dalhousie Medical School, Halifax, Nova Scotia, Canada.
Am J Med Genet A. 2016 Aug;170(8):2012-21. doi: 10.1002/ajmg.a.37769. Epub 2016 Jun 8.
Health-related Quality of Life and the Impact of Childhood Neurologic Disability Scale were collected for 53 patients with CHARGE syndrome aged 13-39 years with a mean academic level of 4th grade. The most prevalent new and ongoing issues included bone health issues, sleep apnea, retinal detachment, anxiety, and aggression. Sleep issues were significantly correlated with anxiety, self-abuse, conduct problems, and autistic-like behaviors. Problems with overall health, behavior, and balance most affected the number of social activities in the individual's life. Sensory impairment most affected relationships with friends. Two contrasting case studies are presented and demonstrate that the quality of life exists on a broad spectrum in CHARGE syndrome, just as its physical features range from mild to very severe. A multitude of factors, including those beyond the physical manifestations, such as anxiety and sleep problems, influence quality of life and are important areas for intervention. © 2016 Wiley Periodicals, Inc.
收集了53名年龄在13至39岁、平均学业水平为四年级的CHARGE综合征患者的健康相关生活质量及儿童神经功能障碍量表影响情况。最常见的新出现和持续存在的问题包括骨骼健康问题、睡眠呼吸暂停、视网膜脱离、焦虑和攻击行为。睡眠问题与焦虑、自我伤害、行为问题和自闭症样行为显著相关。整体健康、行为和平衡方面的问题对个体生活中的社交活动数量影响最大。感觉障碍对与朋友的关系影响最大。呈现了两个对比性的案例研究,表明CHARGE综合征患者的生活质量存在广泛差异,就如同其身体特征从轻度到非常严重一样。许多因素,包括身体表现之外的因素,如焦虑和睡眠问题,都会影响生活质量,并且是重要的干预领域。© 2016威利期刊公司