Netherton syndrome; neuropsychological and psychosocial functioning of child and adult patients and their parents

BACKGROUND: Netherton syndrome is a rare severe skin disease. Clinical experience showed considerable psychosocial burdens among Netherton syndrome patients/families. Their (neuro)psychological functioning has never been investigated. OBJECTIVE: To investigate neuropsychological/psychosocial functio...

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Autores principales: Versteegh, Josette JWM, Dulfer, Karolijn, Stuvel, Kira, Pasmans, Suzanne GMA, Utens, Elisabeth MWJ
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2018
Materias:
Articles
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7583452/
https://www.ncbi.nlm.nih.gov/pubmed/30129381
http://dx.doi.org/10.1177/1359105318790052
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author Versteegh, Josette JWM
Dulfer, Karolijn
Stuvel, Kira
Pasmans, Suzanne GMA
Utens, Elisabeth MWJ
author_facet Versteegh, Josette JWM
Dulfer, Karolijn
Stuvel, Kira
Pasmans, Suzanne GMA
Utens, Elisabeth MWJ
author_sort Versteegh, Josette JWM
collection PubMed
description BACKGROUND: Netherton syndrome is a rare severe skin disease. Clinical experience showed considerable psychosocial burdens among Netherton syndrome patients/families. Their (neuro)psychological functioning has never been investigated. OBJECTIVE: To investigate neuropsychological/psychosocial functioning of Netherton syndrome patients and parents. METHODS: A total of 12 Netherton syndrome patients and/or parents completed neuropsychological tests, semi-structured-interviews, and psychological-questionnaires. RESULTS: Intelligence results showed disharmonic profiles, with below-average scores on processing speed. Neuropsychological problems and unfavorable outcomes on health-related quality of life, illness-appearance-related problems, and negative social consequences among patients/parents were found. Psychopathological (emotional) problems were reported; stigmatization, bullying was common among Netherton syndrome patients. CONCLUSION: Compared with normative data, Netherton syndrome patients showed neuropsychological and psychosocial problems. Standard follow-up is necessary to identify problems at early stage.
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spelling pubmed-75834522020-11-02 Netherton syndrome; neuropsychological and psychosocial functioning of child and adult patients and their parents Versteegh, Josette JWM Dulfer, Karolijn Stuvel, Kira Pasmans, Suzanne GMA Utens, Elisabeth MWJ J Health Psychol Articles BACKGROUND: Netherton syndrome is a rare severe skin disease. Clinical experience showed considerable psychosocial burdens among Netherton syndrome patients/families. Their (neuro)psychological functioning has never been investigated. OBJECTIVE: To investigate neuropsychological/psychosocial functioning of Netherton syndrome patients and parents. METHODS: A total of 12 Netherton syndrome patients and/or parents completed neuropsychological tests, semi-structured-interviews, and psychological-questionnaires. RESULTS: Intelligence results showed disharmonic profiles, with below-average scores on processing speed. Neuropsychological problems and unfavorable outcomes on health-related quality of life, illness-appearance-related problems, and negative social consequences among patients/parents were found. Psychopathological (emotional) problems were reported; stigmatization, bullying was common among Netherton syndrome patients. CONCLUSION: Compared with normative data, Netherton syndrome patients showed neuropsychological and psychosocial problems. Standard follow-up is necessary to identify problems at early stage. SAGE Publications 2018-08-21 2020-11 /pmc/articles/PMC7583452/ /pubmed/30129381 http://dx.doi.org/10.1177/1359105318790052 Text en © The Author(s) 2018 http://www.creativecommons.org/licenses/by-nc/4.0/ This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (http://www.creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Articles
Versteegh, Josette JWM
Dulfer, Karolijn
Stuvel, Kira
Pasmans, Suzanne GMA
Utens, Elisabeth MWJ
Netherton syndrome; neuropsychological and psychosocial functioning of child and adult patients and their parents
title Netherton syndrome; neuropsychological and psychosocial functioning of child and adult patients and their parents
title_full Netherton syndrome; neuropsychological and psychosocial functioning of child and adult patients and their parents
title_fullStr Netherton syndrome; neuropsychological and psychosocial functioning of child and adult patients and their parents
title_full_unstemmed Netherton syndrome; neuropsychological and psychosocial functioning of child and adult patients and their parents
title_short Netherton syndrome; neuropsychological and psychosocial functioning of child and adult patients and their parents
title_sort netherton syndrome; neuropsychological and psychosocial functioning of child and adult patients and their parents
topic Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7583452/
https://www.ncbi.nlm.nih.gov/pubmed/30129381
http://dx.doi.org/10.1177/1359105318790052
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