von Gontard A, Backes M, Laufersweiler-Plass C, Wendland C, Lehmkuhl G, Zerres K, Rudnik-Schöneborn S
Department of Child Psychiatry, University of Cologne, Köln, Germany.
J Child Psychol Psychiatry. 2002 Oct;43(7):949-57. doi: 10.1111/1469-7610.00098.
Chronic illness and mental retardation are both associated with an increased rate of behavioural problems in children and with considerable emotional strain in families. The aim of the study was to analyse and compare the specific effects of two exemplary conditions on familial stress and coping.
Forty-nine boys with Fragile X syndrome (FXS) were compared with 46 boys with Spinal Muscular Atrophy (SMA) and 32 male controls. Intelligence was measured with the RAVEN or K-ABC tests. Psychopathology was assessed with the CBCL questionnaire and a structured psychiatric interview (Kinder-DIPS), parental stress with the QRS, coping with the F-COPES and social support with the F-SOZU questionnaires.
The mean age of the FXS boys was 8.6, of the SMA boys 12.7 and of the controls 11.2 years. The mean IQ was 47 for the FXS, 112 for the SMA and 103 for the control groups. According to the CBCL, 89.8% of the FXS boys, 21.7% of the SMA and 15.7% of the controls had a total score in the borderline or clinical range. The rates were 63.3%, 34.8% and 21.9% for internalising and 67.3%, 10.9% and 18.8% for externalising behaviour, respectively. 81.6% of the FXS and 10.9% of the SMA patients had a DSM-IV or ICD-10 psychiatric diagnosis. The most common were ADHD (FXS: 36) and Separation Anxiety Disorder (SMA: 4). In total, parental stress was significantly higher in the FXS than in the SMA families (and in both compared to controls). There were no major inter-group differences regarding social support and familial coping.
Children with FXS are severely mentally retarded and have a high rate of mainly externalising disorders. Despite good coping abilities and social support, this is associated with high familial stress. The SMA boys, with an intelligence in the upper normal range, are no more deviant than their healthy controls. Parental stress is lower in the SMA families with good coping abilities. In conclusion, families with mentally retarded children are in even greater need of help than those of children with severe chronic illness/physical handicap.
慢性病和智力发育迟缓都与儿童行为问题发生率增加以及家庭中相当大的情感压力有关。本研究的目的是分析和比较两种典型情况对家庭压力和应对方式的具体影响。
将49名患有脆性X综合征（FXS）的男孩与46名患有脊髓性肌萎缩症（SMA）的男孩及32名男性对照组进行比较。通过瑞文测试或考夫曼儿童成套评估测验（K-ABC）测量智力。使用儿童行为量表（CBCL）问卷和结构化精神科访谈（儿童诊断访谈量表，Kinder-DIPS）评估精神病理学，用父母压力量表（QRS）评估父母压力，用家庭应对方式问卷（F-COPES）评估应对方式，用家庭社会支持问卷（F-SOZU）评估社会支持。
FXS组男孩的平均年龄为8.6岁，SMA组男孩为12.7岁，对照组为11.2岁。FXS组的平均智商为47，SMA组为112，对照组为103。根据CBCL，89.8%的FXS男孩、21.7%的SMA男孩和15.7%的对照组男孩总分处于临界或临床范围。内化行为的发生率分别为63.3%、34.8%和21.9%，外化行为的发生率分别为67.3%、10.9%和18.8%。81.6%的FXS患者和10.9%的SMA患者有DSM-IV或ICD-10精神科诊断。最常见的是注意力缺陷多动障碍（FXS组：36例）和分离焦虑障碍（SMA组：4例）。总体而言，FXS家庭中父母的压力显著高于SMA家庭（与对照组相比，两组均如此）。在社会支持和家庭应对方面，组间没有重大差异。
FXS儿童存在严重智力发育迟缓，主要以外化性障碍为主的发生率较高。尽管有良好的应对能力和社会支持，但这仍与高家庭压力相关。智力处于正常上限范围的SMA男孩与健康对照组相比并无更多偏差行为。具有良好应对能力的SMA家庭中父母压力较低。总之，与患有严重慢性病/身体残疾儿童的家庭相比，智力发育迟缓儿童的家庭更需要帮助。
