Cargando…

Quality of Life in Chronic Ketogenic Diet Treatment: The GLUT1DS Population Perspective

Background: Glucose transporter type 1 deficiency syndrome (GLUT1DS) is a rare, genetically determined neurological disorder, for which Ketogenic Diet (KD) represents the gold standard life-long treatment. The aim of this study is to investigate health related quality of life in a well characterized...

Descripción completa

Detalles Bibliográficos
Autores principales: Varesio, Costanza, Pasca, Ludovica, Parravicini, Stefano, Zanaboni, Martina Paola, Ballante, Elena, Masnada, Silvia, Ferraris, Cinzia, Bertoli, Simona, Tagliabue, Anna, Veggiotti, Pierangelo, De Giorgis, Valentina
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6682968/
https://www.ncbi.nlm.nih.gov/pubmed/31330987
http://dx.doi.org/10.3390/nu11071650
_version_ 1783441987437854720
author Varesio, Costanza
Pasca, Ludovica
Parravicini, Stefano
Zanaboni, Martina Paola
Ballante, Elena
Masnada, Silvia
Ferraris, Cinzia
Bertoli, Simona
Tagliabue, Anna
Veggiotti, Pierangelo
De Giorgis, Valentina
author_facet Varesio, Costanza
Pasca, Ludovica
Parravicini, Stefano
Zanaboni, Martina Paola
Ballante, Elena
Masnada, Silvia
Ferraris, Cinzia
Bertoli, Simona
Tagliabue, Anna
Veggiotti, Pierangelo
De Giorgis, Valentina
author_sort Varesio, Costanza
collection PubMed
description Background: Glucose transporter type 1 deficiency syndrome (GLUT1DS) is a rare, genetically determined neurological disorder, for which Ketogenic Diet (KD) represents the gold standard life-long treatment. The aim of this study is to investigate health related quality of life in a well characterized cohort of patients affected by GLUT1DS treated with KD, evaluating factors that can influence patients’ and parents’ quality of life perception. Methods: This is a double center exploratory research study. A postal survey with auto-administrable questionnaires was conducted among 17 subjects (aged 3–22 years) with diagnosis of GLUT1DS, receiving a stable KD treatment for more than 1 year. The Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales was adopted. Clinical variables analyzed in relation to quality of life were frequency of epileptic seizures and movement disorder since KD introduction, presence of intellectual disability (ID), and KD ratio. Results: Quality of life global scores were impaired both in parents’ and children’s perspectives, with a significant concordance. Taking into consideration subscales, the average was 64.17 (range 10–100) for physical functioning, 74.23 (range 30–100) for emotional functioning, 62.64 (range 10–100) for social functioning, and 56 (range 15–92) for school functioning. Conclusions: In patients with GLUT1DS the quality of life perception is comparable to that of other patients with chronic disease. In our sample, the presence of movement disorder seems to be a crucial element in quality of life perception.
format Online
Article
Text
id pubmed-6682968
institution National Center for Biotechnology Information
language English
publishDate 2019
publisher MDPI
record_format MEDLINE/PubMed
spelling pubmed-66829682019-08-09 Quality of Life in Chronic Ketogenic Diet Treatment: The GLUT1DS Population Perspective Varesio, Costanza Pasca, Ludovica Parravicini, Stefano Zanaboni, Martina Paola Ballante, Elena Masnada, Silvia Ferraris, Cinzia Bertoli, Simona Tagliabue, Anna Veggiotti, Pierangelo De Giorgis, Valentina Nutrients Article Background: Glucose transporter type 1 deficiency syndrome (GLUT1DS) is a rare, genetically determined neurological disorder, for which Ketogenic Diet (KD) represents the gold standard life-long treatment. The aim of this study is to investigate health related quality of life in a well characterized cohort of patients affected by GLUT1DS treated with KD, evaluating factors that can influence patients’ and parents’ quality of life perception. Methods: This is a double center exploratory research study. A postal survey with auto-administrable questionnaires was conducted among 17 subjects (aged 3–22 years) with diagnosis of GLUT1DS, receiving a stable KD treatment for more than 1 year. The Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales was adopted. Clinical variables analyzed in relation to quality of life were frequency of epileptic seizures and movement disorder since KD introduction, presence of intellectual disability (ID), and KD ratio. Results: Quality of life global scores were impaired both in parents’ and children’s perspectives, with a significant concordance. Taking into consideration subscales, the average was 64.17 (range 10–100) for physical functioning, 74.23 (range 30–100) for emotional functioning, 62.64 (range 10–100) for social functioning, and 56 (range 15–92) for school functioning. Conclusions: In patients with GLUT1DS the quality of life perception is comparable to that of other patients with chronic disease. In our sample, the presence of movement disorder seems to be a crucial element in quality of life perception. MDPI 2019-07-19 /pmc/articles/PMC6682968/ /pubmed/31330987 http://dx.doi.org/10.3390/nu11071650 Text en © 2019 by the authors. 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 (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Varesio, Costanza
Pasca, Ludovica
Parravicini, Stefano
Zanaboni, Martina Paola
Ballante, Elena
Masnada, Silvia
Ferraris, Cinzia
Bertoli, Simona
Tagliabue, Anna
Veggiotti, Pierangelo
De Giorgis, Valentina
Quality of Life in Chronic Ketogenic Diet Treatment: The GLUT1DS Population Perspective
title Quality of Life in Chronic Ketogenic Diet Treatment: The GLUT1DS Population Perspective
title_full Quality of Life in Chronic Ketogenic Diet Treatment: The GLUT1DS Population Perspective
title_fullStr Quality of Life in Chronic Ketogenic Diet Treatment: The GLUT1DS Population Perspective
title_full_unstemmed Quality of Life in Chronic Ketogenic Diet Treatment: The GLUT1DS Population Perspective
title_short Quality of Life in Chronic Ketogenic Diet Treatment: The GLUT1DS Population Perspective
title_sort quality of life in chronic ketogenic diet treatment: the glut1ds population perspective
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6682968/
https://www.ncbi.nlm.nih.gov/pubmed/31330987
http://dx.doi.org/10.3390/nu11071650
work_keys_str_mv AT varesiocostanza qualityoflifeinchronicketogenicdiettreatmenttheglut1dspopulationperspective
AT pascaludovica qualityoflifeinchronicketogenicdiettreatmenttheglut1dspopulationperspective
AT parravicinistefano qualityoflifeinchronicketogenicdiettreatmenttheglut1dspopulationperspective
AT zanabonimartinapaola qualityoflifeinchronicketogenicdiettreatmenttheglut1dspopulationperspective
AT ballanteelena qualityoflifeinchronicketogenicdiettreatmenttheglut1dspopulationperspective
AT masnadasilvia qualityoflifeinchronicketogenicdiettreatmenttheglut1dspopulationperspective
AT ferrariscinzia qualityoflifeinchronicketogenicdiettreatmenttheglut1dspopulationperspective
AT bertolisimona qualityoflifeinchronicketogenicdiettreatmenttheglut1dspopulationperspective
AT tagliabueanna qualityoflifeinchronicketogenicdiettreatmenttheglut1dspopulationperspective
AT veggiottipierangelo qualityoflifeinchronicketogenicdiettreatmenttheglut1dspopulationperspective
AT degiorgisvalentina qualityoflifeinchronicketogenicdiettreatmenttheglut1dspopulationperspective