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CC BY-NC-ND 4.0 · Journal of Child Science 2018; 08(01): e124-e127
DOI: 10.1055/s-0038-1667348
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Assessment and Management of Over-Activity and Sleep Disorder in Mucopolysaccharidoses

Dougal Julian Hare
1   School of Psychology, Cardiff University, Cardiff, Wales, United Kingdom
,
Louise Mahon
2   Manchester Mental Health and Social Care NHS Trust, Manchester, United Kingdom
,
Stewart Rust
3   Department of Paediatric Psychosocialogy, Royal Manchester Children's Hospital, Manchester, United Kingdom
› Author Affiliations
Further Information

Publication History

25 October 2017

18 April 2018

Publication Date:
03 October 2018 (online)

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Abstract

There is a growing awareness, based on both research and clinical studies, that abnormal sleep and circadian functioning are associated with the various forms of mucopolysaccharidoses (MPS), with sleep respiratory problems seemingly common in many forms of MPS. In the case of MPS III, there is evidence of syndrome-specific sleep disturbance related to an abnormal and possibly unique circadian rhythm. The presence of such patterns of sleep and activity has detrimental consequences for both the person with MPS and also invariably for her/his family and carers. On the basis of current evidence, routine assessment of sleep functioning is clearly warranted for all children with MPS III together with appropriate medical and behavioral interventions as required.

Keywords

mucopolysaccharides - Sanfilippo syndrome - sleep - circadian - activity
 

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