Behaviour in mucopolysaccharide disorders

Title

Behaviour in mucopolysaccharide disorders

Creator

Bax M C; Colville G A

Identifier

Publisher

Archives of Disease in Childhood

Date

1995

Subject

Data Collection; Parents; Humans; Prevalence; Family Health; Child Preschool; Self-Help Groups; Mucopolysaccharidosis III/psychology; Mucopolysaccharidoses/complications; Aggression; Child Behavior Disorders/complications; Mucopolysaccharidosis II/psychology; Sleep Disorders/complications; Q3 Literature Search; child; female; male; behavior; sleep disturbance; MPSI; MPSII; MPSIII; trajectory; characteristics; destructiveness; restlessness; aggressiveness

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.

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).

Citation

Bax M C; Colville G A, “Behaviour in mucopolysaccharide disorders,” Pediatric Palliative Care Library, accessed August 3, 2021, https://pedpalascnetlibrary.omeka.net/items/show/16585.

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