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桑菲利波综合征中与认知、行为和运动障碍相关的发育和退化模式:一项流行病学研究。

Developmental and degenerative patterns associated with cognitive, behavioural and motor difficulties in the Sanfilippo syndrome: an epidemiological study.

作者信息

Nidiffer F D, Kelly T E

出版信息

J Ment Defic Res. 1983 Sep;27 (Pt 3):185-203. doi: 10.1111/j.1365-2788.1983.tb00291.x.

DOI:10.1111/j.1365-2788.1983.tb00291.x
PMID:6415286
Abstract

The results of a survey given to parents with Sanfilippo syndrome (MPS III) children (n = 30) are described with an emphasis in several areas. Developmental and degenerative patterns are outlined in detail and contrasted with developmental milestones of normal children. An essentially uniform pattern of behaviour problems was discovered and is discussed along with the efficacy of pharmacological and non-pharmacological approach to remediation. Finally, practical suggestions are offered for physicians and parents which are encountered by the daily management problems of children with this disorder.

摘要

本文描述了一项针对患有Sanfilippo综合征(黏多糖贮积症III型,MPS III)患儿的家长(n = 30)的调查结果,重点关注了几个方面。详细概述了发育和退化模式,并与正常儿童的发育里程碑进行了对比。发现了一种基本一致的行为问题模式,并讨论了药物和非药物治疗方法的疗效。最后,针对患有这种疾病的儿童日常管理问题给医生和家长提供了实用建议。

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