An investigation of the middle and latebehavioural phenotypes of Mucopolysaccharidosis Type-III
health sciences; digital collections; electronic information; information dissemination; life sciences; medical research; scientific information; behavioral problems; MPS III; trajectory; characteristics; challenging behaviors
PubMed Central Canada (PMC Canada) provides free access to a stable and permanent online digital archive of full-text, peer-reviewed health and life sciences research publications. It builds on PubMed Central (PMC), the U.S. National Institutes of Health (NIH) free digital archive of biomedical and life sciences journal literature and is a member of the broader PMC International (PMCI) network of e-repositories.
Cross E M; Grant S; Jones S; Bigger B W; Wraith J E; Mahon L V; Lomax M; Hare D J; Canada Government of Canada National Research Council
Journal of Neurodevelopmental Disorders
2014
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).
<a href="http://doi.org/10.1186/1866-1955-6-46" target="_blank" rel="noreferrer noopener">10.1186/1866-1955-6-46</a>
Assessment of sleep in children with mucopolysaccharidosis type III
Actigraphy; Adolescent; Case-Control Studies; Child; Preschool; Female; Humans; Male; Melatonin/me [Metabolism]; Mucopolysaccharidosis III/pp [Physiopathology]; Sleep/ph [Physiology]; Time Factors; JL5DK93RCL (Melatonin); sleep disturbance/disorders; MPSIIIA; MPSIIIB; trajectory; characteristics
Sleep disturbances are prevalent in mucopolysaccharidosis Type III (MPS III), yet there is a lack of objective, ecologically valid evidence detailing sleep quantity, quality or circadian system. Eight children with MPS III and eight age-matched typically developing children wore an actigraph for 7-10 days/nights. Saliva samples were collected at three time-points on two separate days, to permit analysis of endogenous melatonin levels. Parents completed a sleep questionnaire and a daily sleep diary. Actigraphic data revealed that children with MPS III had significantly longer sleep onset latencies and greater daytime sleep compared to controls, but night-time sleep duration did not differ between groups. In the MPS III group, sleep efficiency declined, and sleep onset latency increased, with age. Questionnaire responses showed that MPS III patients had significantly more sleep difficulties in all domains compared to controls. Melatonin concentrations showed an alteration in the circadian system in MPS III, which suggests that treatment for sleep problems should attempt to synchronise the sleep-wake cycle to a more regular pattern. Actigraphy was tolerated by children and this monitoring device can be recommended as a measure of treatment success in research and clinical practice.
Mahon L V; Lomax M; Grant S; Cross E; Hare D J; Wraith J E; Jones S; Bigger B; Langford-Smith K; Canal M
PLoS ONE
2014
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).
<a href="http://doi.org/10.1371/journal.pone.0084128" target="_blank" rel="noreferrer noopener">10.1371/journal.pone.0084128</a>