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Journal article
Psychosocial functioning and determinants of the health-related quality of life in children with neurofibromatosis type 1 and cognitive impairments
Journal of neuro-oncology, Vol.First online
05/06/2025
PMID: 40471421
Appears in Recent Faculty of Health Publications
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Source: InCites
Abstract
Purpose: Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic condition associated with cutaneous and neoplastic manifestations and other physical manifestations, as well as cognitive, psychosocial, and behavioural difficulties. NF1 negatively impacts the health-related quality of life (HRQoL) of children. There is limited evidence regarding the determinants of HRQoL of children with NF1. The aim of this study was to (i) compare the HRQoL of children with NF1 and cognitive impairments to published data of healthy children and children with cancer and (ii) identify specific determinants of child and parent-proxy reports of psychosocial HRQoL.
Methods: Children with NF1 and cognitive impairments (n = 135, 8-15 years 11 months) and their parents completed standardized measures assessing children's HRQoL, behavioral and emotional functioning. Children completed a brief intelligence test. Correlations and multiple linear regressions were conducted to identify determinants of psychosocial HRQoL.
Results: Children with NF1 had significantly poorer HRQoL for all domains than published data of healthy children and significantly poorer HRQoL for Psychosocial Health, School and Social Functioning than published data of children with cancer. For child self-report, attention problems and increased social stress predicted their psychosocial HRQoL. For parent-proxy reports, activities of daily living and depression were significant predictors of children's psychosocial HRQoL. Social stress and depression were the strongest predictors of Psychosocial HRQoL.
Conclusion: Routine screening and early identification of depressive symptoms and interventions that promote social support, coping and resiliency may improve the HRQoL of children with NF1.
Details
- Title
- Psychosocial functioning and determinants of the health-related quality of life in children with neurofibromatosis type 1 and cognitive impairments
- Creators
- Belinda Barton - Southern Cross UniversityPamela L. Wolters - National Institutes of HealthKarin S. Walsh - Children’s National Hospital (USA, Washington)Nicole J. Ullrich - Boston Children's HospitalTena Rosser - Children's Hospital of Los AngelesJames Tonsgard - University of Chicago Medicine Comer Children’s Hospital (USA, Chicago)David Viskochil - University of UtahElizabeth Schorry - Cincinnati Children's Hospital Medical CenterLaura J. Klesse - The University of Texas Southwestern Medical CenterMichael J. Fisher - Children's Hospital of PhiladelphiaDavid H. Gutmann - Washington University School of Medicine (USA, St Louis)Roger J. Packer - Children’s National Hospital (USA, Washington)Bruce Korf - University of Alabama at BirminghamMaria T. Acosta - National Human Genome Research InstituteKathryn N. North - The Royal Children’s Hospital (Australia, Parkville)Jonathan M. Payne - The Royal Children’s Hospital (Australia, Parkville)NF Clinical Trials Consortium
- Publication Details
- Journal of neuro-oncology, Vol.First online
- Publisher
- Springer Nature
- Number of pages
- 12
- Grant note
- Open Access funding enabled and organized by CAUL and its Member Institutions. This study was primarily funded by the US Army Medical Research and Materiel Command, Office of the Congressionally Directed Medical Research Programs, Department of Defense Neurofibromatosis Research Program (Grant Number: W81XWH-05-1-0615)
- Identifiers
- 991013291354202368
- Copyright
- © The Author(s) 2025
- Academic Unit
- Human Sciences; Faculty of Health
- Language
- English
- Resource Type
- Journal article