Behaviour in mucopolysaccharide disorders

Title

Behaviour in mucopolysaccharide disorders

Creator

Bax MC; Colville GA

Identifier

Publisher

Archives Of Disease In Childhood

Date

1995

Subject

Child; Female; Humans; Male; Prevalence; Data Collection; Parents; Family Health; Self-Help Groups; Preschool; Q3 Literature Search; Mucopolysaccharidoses/complications; Mucopolysaccharidosis III/psychology; Aggression; Child Behavior Disorders/complications; Mucopolysaccharidosis II/psychology; Sleep Disorders/complications

Description

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.
1995

Rights

Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).

Type

Journal Article

Citation List Month

Backlog

Pages

77-81

Issue

1

Volume

73

Citation

Bax MC; Colville GA, “Behaviour in mucopolysaccharide disorders,” Pediatric Palliative Care Library, accessed September 24, 2021, https://pedpalascnetlibrary.omeka.net/items/show/11906.

Social Bookmarking