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dc.contributor.authorGrant, Nathan
dc.date.accessioned2021-04-08T15:32:22Z
dc.date.available2021-04-08T15:32:22Z
dc.date.issued2021-04-08
dc.date.submitted2020-11-26
dc.identifier.others13023-021-01767-8
dc.identifier.other1767
dc.identifier.urihttps://www.repository.cam.ac.uk/handle/1810/319586
dc.description.abstractAbstract: The mucopolysaccharidoses (MPS) are a group of rare, genetic, lysosomal storage disorders characterized by progressive, multisystemic accumulation of glycosaminoglycans. Individuals with severe MPS often present with significant neurological involvement and may exhibit challenging behaviors, including hyperactivity, aggression, and sleep disturbance. These behaviors can cause adverse outcomes and necessitate the development of specific measures to support affected families. Through an analysis of the results reported by Hoffmann et al. in their recent study, this letter outlines important factors that must be considered when evaluating the impact of challenging behaviors associated with MPS, including treatment history, age, sibling and family relationships, the feasibility of daily caregiving, and caregiver burden. These recommendations can help guide future studies to identify the most effective coping strategies to support families of people with MPS who have challenging behaviors.
dc.languageen
dc.publisherBioMed Central
dc.subjectLetter to the Editor
dc.subjectLysosomal storage diseases
dc.subjectMucopolysaccharidoses
dc.subjectMPS
dc.subjectChallenging behavior
dc.subjectAggression
dc.subjectCaregivers
dc.subjectSiblings
dc.subjectCoping
dc.subjectSupport
dc.titleEvaluating strategies to manage and endure challenging behaviors in mucopolysaccharidoses
dc.typeOther
dc.date.updated2021-04-08T15:32:21Z
prism.issueIdentifier1
prism.publicationNameOrphanet Journal of Rare Diseases
prism.volume16
dc.identifier.doi10.17863/CAM.66706
dcterms.dateAccepted2021-03-01
rioxxterms.versionofrecord10.1186/s13023-021-01767-8
rioxxterms.versionVoR
rioxxterms.licenseref.urihttp://creativecommons.org/licenses/by/4.0/
dc.contributor.orcidGrant, Nathan [0000-0001-9252-4896]
dc.identifier.eissn1750-1172


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