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Clinical spectrum and the comorbidities of Dravet syndrome in Taiwan and the possible molecular mechanisms

Dravet syndrome (DS) is an uncommon epilepsy syndrome that may negatively affect the patients and their caregivers. However, reliable and valid measures of its impact on caregivers and the characteristics of patients with DS in Taiwan are lacking. This study aimed to describe the characteristics of...

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Autores principales: Huang, Chia-Hsuan, Hung, Pi-Lien, Fan, Pi-Chuan, Lin, Kuang-Lin, Hsu, Ting-Rong, Chou, I-Jun, Ho, Che-Sheng, Chou, I-Ching, Lin, Wei-Sheng, Lee, Inn-Chi, Fan, Hueng-Chuen, Chen, Shyi-Jou, Liang, Jao-Shwann, Tu, Yi-Fang, Chang, Tung-Ming, Hu, Su-Ching, Wong, Lee-Chin, Hung, Kun-Long, Lee, Wang-Tso
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8511137/
https://www.ncbi.nlm.nih.gov/pubmed/34642351
http://dx.doi.org/10.1038/s41598-021-98517-4
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author Huang, Chia-Hsuan
Hung, Pi-Lien
Fan, Pi-Chuan
Lin, Kuang-Lin
Hsu, Ting-Rong
Chou, I-Jun
Ho, Che-Sheng
Chou, I-Ching
Lin, Wei-Sheng
Lee, Inn-Chi
Fan, Hueng-Chuen
Chen, Shyi-Jou
Liang, Jao-Shwann
Tu, Yi-Fang
Chang, Tung-Ming
Hu, Su-Ching
Wong, Lee-Chin
Hung, Kun-Long
Lee, Wang-Tso
author_facet Huang, Chia-Hsuan
Hung, Pi-Lien
Fan, Pi-Chuan
Lin, Kuang-Lin
Hsu, Ting-Rong
Chou, I-Jun
Ho, Che-Sheng
Chou, I-Ching
Lin, Wei-Sheng
Lee, Inn-Chi
Fan, Hueng-Chuen
Chen, Shyi-Jou
Liang, Jao-Shwann
Tu, Yi-Fang
Chang, Tung-Ming
Hu, Su-Ching
Wong, Lee-Chin
Hung, Kun-Long
Lee, Wang-Tso
author_sort Huang, Chia-Hsuan
collection PubMed
description Dravet syndrome (DS) is an uncommon epilepsy syndrome that may negatively affect the patients and their caregivers. However, reliable and valid measures of its impact on caregivers and the characteristics of patients with DS in Taiwan are lacking. This study aimed to describe the characteristics of patients with DS and concerns of their caregivers and establish a baseline frequency of disease characteristics using a cross-sectional survey in Taiwan. We assessed the caregivers of patients with DS using an online anonymous questionnaire. The seizure frequency decreased with age, although lacking statistical significance. Vaccines show no influence on the condition of patients with DS. Our findings revealed the highest impact on the domains affecting the caregivers’ daily life, including additional household tasks, symptom observation, further medical plan, and financial issues. Caregivers also expressed concerns regarding the lack of independence/constant care, seizure control, speech/communication, and impacts on siblings because of long-term care of the patients in parents’ absence. Our findings highlight the significant effects of caring for a child with DS on the lives of their caregivers in Taiwan; these findings will help raise awareness regarding the needs of these families. Furthermore, we discussed the possible pathophysiological mechanisms of associated comorbidities.
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spelling pubmed-85111372021-10-14 Clinical spectrum and the comorbidities of Dravet syndrome in Taiwan and the possible molecular mechanisms Huang, Chia-Hsuan Hung, Pi-Lien Fan, Pi-Chuan Lin, Kuang-Lin Hsu, Ting-Rong Chou, I-Jun Ho, Che-Sheng Chou, I-Ching Lin, Wei-Sheng Lee, Inn-Chi Fan, Hueng-Chuen Chen, Shyi-Jou Liang, Jao-Shwann Tu, Yi-Fang Chang, Tung-Ming Hu, Su-Ching Wong, Lee-Chin Hung, Kun-Long Lee, Wang-Tso Sci Rep Article Dravet syndrome (DS) is an uncommon epilepsy syndrome that may negatively affect the patients and their caregivers. However, reliable and valid measures of its impact on caregivers and the characteristics of patients with DS in Taiwan are lacking. This study aimed to describe the characteristics of patients with DS and concerns of their caregivers and establish a baseline frequency of disease characteristics using a cross-sectional survey in Taiwan. We assessed the caregivers of patients with DS using an online anonymous questionnaire. The seizure frequency decreased with age, although lacking statistical significance. Vaccines show no influence on the condition of patients with DS. Our findings revealed the highest impact on the domains affecting the caregivers’ daily life, including additional household tasks, symptom observation, further medical plan, and financial issues. Caregivers also expressed concerns regarding the lack of independence/constant care, seizure control, speech/communication, and impacts on siblings because of long-term care of the patients in parents’ absence. Our findings highlight the significant effects of caring for a child with DS on the lives of their caregivers in Taiwan; these findings will help raise awareness regarding the needs of these families. Furthermore, we discussed the possible pathophysiological mechanisms of associated comorbidities. Nature Publishing Group UK 2021-10-12 /pmc/articles/PMC8511137/ /pubmed/34642351 http://dx.doi.org/10.1038/s41598-021-98517-4 Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) .
spellingShingle Article
Huang, Chia-Hsuan
Hung, Pi-Lien
Fan, Pi-Chuan
Lin, Kuang-Lin
Hsu, Ting-Rong
Chou, I-Jun
Ho, Che-Sheng
Chou, I-Ching
Lin, Wei-Sheng
Lee, Inn-Chi
Fan, Hueng-Chuen
Chen, Shyi-Jou
Liang, Jao-Shwann
Tu, Yi-Fang
Chang, Tung-Ming
Hu, Su-Ching
Wong, Lee-Chin
Hung, Kun-Long
Lee, Wang-Tso
Clinical spectrum and the comorbidities of Dravet syndrome in Taiwan and the possible molecular mechanisms
title Clinical spectrum and the comorbidities of Dravet syndrome in Taiwan and the possible molecular mechanisms
title_full Clinical spectrum and the comorbidities of Dravet syndrome in Taiwan and the possible molecular mechanisms
title_fullStr Clinical spectrum and the comorbidities of Dravet syndrome in Taiwan and the possible molecular mechanisms
title_full_unstemmed Clinical spectrum and the comorbidities of Dravet syndrome in Taiwan and the possible molecular mechanisms
title_short Clinical spectrum and the comorbidities of Dravet syndrome in Taiwan and the possible molecular mechanisms
title_sort clinical spectrum and the comorbidities of dravet syndrome in taiwan and the possible molecular mechanisms
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8511137/
https://www.ncbi.nlm.nih.gov/pubmed/34642351
http://dx.doi.org/10.1038/s41598-021-98517-4
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