Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Behaviour in mucopolysaccharide disorders

M C Bax1, G A Colville

  • 1Academic Department of Child Health, Chelsea and Westminster Hospital, London.

Archives of Disease in Childhood
|July 1, 1995
PubMed
Summary
This summary is machine-generated.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

A survey of moral distress in staff working in intensive care in the UK.

Journal of the Intensive Care Society·2019
Same author

Mothers' recollections of the Paediatric Intensive Care Unit: associations with psychopathology and views on follow up.

Intensive & critical care nursing·2005
Same author

Psychological management of two cases of self injury on the paediatric intensive care unit.

Archives of disease in childhood·2003
Same author

Adolescence and after.

Developmental medicine and child neurology·2001
Same author

Unsolved problems.

Developmental medicine and child neurology·1997
Same author

Sexuality disability and abuse. Research into practice?

European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie·1996
Same journal

Protecting adolescent confidentiality in the digital age: a global call for adolescent-informed electronic health records.

Archives of disease in childhood·2026
Same journal

Diagnostic accuracy study assessing the ability of paediatric asthma scores to predict admission following initial emergency department bronchodilator therapy: a Clinical Asthma Scoring systems in Paediatric Emergency (CASPER) study.

Archives of disease in childhood·2026
Same journal

Artificial intelligence for child health: current capabilities and the next frontier.

Archives of disease in childhood·2026
Same journal

Troubled origins and lasting impact of the first insulin injection.

Archives of disease in childhood·2026
Same journal

Paediatric readiness assessment tools in emergency care: a scoping review.

Archives of disease in childhood·2026
Same journal

Building a paediatric workforce to deliver the NHS prevention agenda: time for paediatric public health medicine?

Archives of disease in childhood·2026
See all related articles

Behavioral issues are common in children with mucopolysaccharide disorders, especially Sanfilippo and Hunter diseases. Parents need more support managing these challenges, which significantly impact families.

Area of Science:

  • Pediatric Psychology
  • Rare Genetic Disorders
  • Behavioral Science

Background:

  • Mucopolysaccharide disorders (MPS) are a group of rare genetic conditions.
  • Behavioral problems are frequently observed in children with MPS, but their nature and prevalence require further investigation.
  • Understanding these behavioral challenges is crucial for providing adequate support to affected children and their families.

Purpose of the Study:

  • To investigate the nature and prevalence of behavioral problems in children with mucopolysaccharide disorders.
  • To identify specific behavioral issues and affected subtypes and age groups.
  • To assess the level of support received by parents for managing these behaviors.

Main Methods:

  • A study involving 258 children diagnosed with mucopolysaccharide disorders.

Related Experiment Videos

  • Data collection through postal questionnaires, supplemented by home visits to 42 families.
  • Discussions with families at Society for Mucopolysaccharide Diseases meetings.

    • Main Results:

    • High rates of behavioral problems were identified, particularly in children aged 5–9 years with Sanfilippo's and Hunter's disease.
    • Common issues included destructiveness, restlessness, and aggressiveness.
    • Sleep problems affected 66% of children across all subtypes.

    Conclusions:

    • Behavioral problems are a primary characteristic of mucopolysaccharide disorders.
    • These challenges impose a significant strain on families.
    • There is an urgent need for improved services and support systems for families managing these behaviors.