Bax M C, Colville G A
Academic Department of Child Health, Chelsea and Westminster Hospital, London.
Arch Dis Child. 1995 Jul;73(1):77-81. doi: 10.1136/adc.73.1.77.
This paper reports a study of the nature and prevalence of behaviour problems in 258 children with mucopolysaccharide disorders. Questionnaire data obtained through the post was supplemented by home visits to 42 families in the sample and by regular discussions with families at meetings of the Society for Mucopolysaccharide Diseases. High rates of behaviour problems were found, particularly in children with Sanfilippo's and Hunter's disease aged 5 to 9 years. These included destructiveness, restlessness, and aggressiveness. Sleep problems were common across subtypes with an overall prevalence of 66%. Parents reported that they received little or no support in the management of these difficult behaviours. It is concluded that behaviour problems are a primary feature of the mucopolysaccharide disorders and place a major strain on families. Services to help families cope with these problems are urgently needed.
本文报告了一项针对258名黏多糖病患儿行为问题的性质及普遍性的研究。通过邮寄问卷收集的数据，辅以对样本中42个家庭的家访以及在黏多糖病协会会议上与这些家庭的定期讨论。研究发现行为问题发生率很高，尤其是在5至9岁的桑菲利波氏病和亨特氏病患儿中。这些行为问题包括破坏性、烦躁不安和攻击性。睡眠问题在各亚型中都很常见，总体患病率为66%。家长们表示，在处理这些棘手行为时，他们几乎没有得到什么支持。研究得出结论，行为问题是黏多糖病的主要特征，给家庭带来了巨大压力。迫切需要提供帮助家庭应对这些问题的服务。
