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Social Responsiveness and Psychosocial Functioning in Adults with Prader–Willi Syndrome

Although various studies have investigated symptoms of autism spectrum disorder (ASD) in Prader–Willi syndrome (PWS), little is known about the consequences of these symptoms, especially in psychosocial function. We aimed to explore ASD symptoms in adults with PWS with special attention to psychosoc...

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Autores principales: Fernández-Lafitte, Meritxell, Cobo, Jesus, Coronas, Ramon, Parra, Isabel, Oliva, Joan Carles, Àlvarez, Aida, Esteba-Castillo, Susanna, Giménez-Palop, Olga, Palao, Diego J., Caixàs, Assumpta
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8911114/
https://www.ncbi.nlm.nih.gov/pubmed/35268524
http://dx.doi.org/10.3390/jcm11051433
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author Fernández-Lafitte, Meritxell
Cobo, Jesus
Coronas, Ramon
Parra, Isabel
Oliva, Joan Carles
Àlvarez, Aida
Esteba-Castillo, Susanna
Giménez-Palop, Olga
Palao, Diego J.
Caixàs, Assumpta
author_facet Fernández-Lafitte, Meritxell
Cobo, Jesus
Coronas, Ramon
Parra, Isabel
Oliva, Joan Carles
Àlvarez, Aida
Esteba-Castillo, Susanna
Giménez-Palop, Olga
Palao, Diego J.
Caixàs, Assumpta
author_sort Fernández-Lafitte, Meritxell
collection PubMed
description Although various studies have investigated symptoms of autism spectrum disorder (ASD) in Prader–Willi syndrome (PWS), little is known about the consequences of these symptoms, especially in psychosocial function. We aimed to explore ASD symptoms in adults with PWS with special attention to psychosocial functionality. This cross-sectional study included 26 adults (15 women) with PWS who attended a reference unit for rare diseases. Participants’ primary caregivers completed the Social Responsiveness Scale (SRS), and clinicians assessed multidimensional functioning with the Personal and Social Performance Scale (PSP). Impaired social responsiveness was identified in 20 (76.9%) participants, and manifest to marked difficulties in social functioning were identified in 13 (50%). Participants with impaired social responsiveness (SRS ≥ 60) had significantly worse scores in functionality measured with the PSP (U = 12.5; p = 0.009) and with three of the four PSP main areas. Moreover, scores for the Social Cognition domain of the SRS correlated positively with the Socially useful activities (p < 0.05) and Personal and social relationships (p < 0.01) main areas of the PSP. These results suggest that difficulties in social skills should be assessed in all psychosocial evaluations of patients with PWS.
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spelling pubmed-89111142022-03-11 Social Responsiveness and Psychosocial Functioning in Adults with Prader–Willi Syndrome Fernández-Lafitte, Meritxell Cobo, Jesus Coronas, Ramon Parra, Isabel Oliva, Joan Carles Àlvarez, Aida Esteba-Castillo, Susanna Giménez-Palop, Olga Palao, Diego J. Caixàs, Assumpta J Clin Med Article Although various studies have investigated symptoms of autism spectrum disorder (ASD) in Prader–Willi syndrome (PWS), little is known about the consequences of these symptoms, especially in psychosocial function. We aimed to explore ASD symptoms in adults with PWS with special attention to psychosocial functionality. This cross-sectional study included 26 adults (15 women) with PWS who attended a reference unit for rare diseases. Participants’ primary caregivers completed the Social Responsiveness Scale (SRS), and clinicians assessed multidimensional functioning with the Personal and Social Performance Scale (PSP). Impaired social responsiveness was identified in 20 (76.9%) participants, and manifest to marked difficulties in social functioning were identified in 13 (50%). Participants with impaired social responsiveness (SRS ≥ 60) had significantly worse scores in functionality measured with the PSP (U = 12.5; p = 0.009) and with three of the four PSP main areas. Moreover, scores for the Social Cognition domain of the SRS correlated positively with the Socially useful activities (p < 0.05) and Personal and social relationships (p < 0.01) main areas of the PSP. These results suggest that difficulties in social skills should be assessed in all psychosocial evaluations of patients with PWS. MDPI 2022-03-05 /pmc/articles/PMC8911114/ /pubmed/35268524 http://dx.doi.org/10.3390/jcm11051433 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Fernández-Lafitte, Meritxell
Cobo, Jesus
Coronas, Ramon
Parra, Isabel
Oliva, Joan Carles
Àlvarez, Aida
Esteba-Castillo, Susanna
Giménez-Palop, Olga
Palao, Diego J.
Caixàs, Assumpta
Social Responsiveness and Psychosocial Functioning in Adults with Prader–Willi Syndrome
title Social Responsiveness and Psychosocial Functioning in Adults with Prader–Willi Syndrome
title_full Social Responsiveness and Psychosocial Functioning in Adults with Prader–Willi Syndrome
title_fullStr Social Responsiveness and Psychosocial Functioning in Adults with Prader–Willi Syndrome
title_full_unstemmed Social Responsiveness and Psychosocial Functioning in Adults with Prader–Willi Syndrome
title_short Social Responsiveness and Psychosocial Functioning in Adults with Prader–Willi Syndrome
title_sort social responsiveness and psychosocial functioning in adults with prader–willi syndrome
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8911114/
https://www.ncbi.nlm.nih.gov/pubmed/35268524
http://dx.doi.org/10.3390/jcm11051433
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