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The prevalence and impact of orthostatic intolerance in young women across the hypermobility spectrum

Orthostatic intolerance (OI) is frequently reported in young women with generalized hypermobility spectrum disorder (G‐HSD) and hypermobile EDS (hEDS). However, it remains currently unclear whether OI is a comorbidity or fundamental part of the pathophysiology of G‐HSD or hEDS. This study investigat...

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Autores principales: Peebles, Karen C., Tan, Isabella, Butlin, Mark, Collins, Felicity, Tofts, Louise, Avolio, Alberto P., Pacey, Verity
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley & Sons, Inc. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9305471/
https://www.ncbi.nlm.nih.gov/pubmed/35224842
http://dx.doi.org/10.1002/ajmg.a.62705
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author Peebles, Karen C.
Tan, Isabella
Butlin, Mark
Collins, Felicity
Tofts, Louise
Avolio, Alberto P.
Pacey, Verity
author_facet Peebles, Karen C.
Tan, Isabella
Butlin, Mark
Collins, Felicity
Tofts, Louise
Avolio, Alberto P.
Pacey, Verity
author_sort Peebles, Karen C.
collection PubMed
description Orthostatic intolerance (OI) is frequently reported in young women with generalized hypermobility spectrum disorder (G‐HSD) and hypermobile EDS (hEDS). However, it remains currently unclear whether OI is a comorbidity or fundamental part of the pathophysiology of G‐HSD or hEDS. This study investigated the prevalence and impact of OI in young women across the hypermobility spectrum. Forty‐five women (14–30 years, 15 controls, 15 G‐HSD, and 15 hEDS) undertook a head‐up tilt (HUT) and active stand test. Postural Orthostatic Tachycardia Syndrome (POTS) and Orthostatic Hypotension (OH) were assessed using age‐related criteria. Autonomic dysfunction and quality‐of‐life questionnaires were also completed. The prevalence of POTS was higher in women with G‐HSD than hEDS and control groups during HUT (43% vs. 7% and 7%, respectively, p < 0.05), but similar between groups during the active stand (47%, 27%, and 13% for G‐HSD, hEDS, and control, respectively). No participants had OH. hEDS and G‐HSD participants reported more severe orthostatic symptoms and poorer quality of life than controls. Although POTS was observed in hypermobile participants, there is no conclusive evidence that its prevalence differed between groups due to differences between the HUT and active stand assessments. Nevertheless, OI and broader autonomic dysfunction impacted on their quality of life.
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spelling pubmed-93054712022-07-28 The prevalence and impact of orthostatic intolerance in young women across the hypermobility spectrum Peebles, Karen C. Tan, Isabella Butlin, Mark Collins, Felicity Tofts, Louise Avolio, Alberto P. Pacey, Verity Am J Med Genet A Original Articles Orthostatic intolerance (OI) is frequently reported in young women with generalized hypermobility spectrum disorder (G‐HSD) and hypermobile EDS (hEDS). However, it remains currently unclear whether OI is a comorbidity or fundamental part of the pathophysiology of G‐HSD or hEDS. This study investigated the prevalence and impact of OI in young women across the hypermobility spectrum. Forty‐five women (14–30 years, 15 controls, 15 G‐HSD, and 15 hEDS) undertook a head‐up tilt (HUT) and active stand test. Postural Orthostatic Tachycardia Syndrome (POTS) and Orthostatic Hypotension (OH) were assessed using age‐related criteria. Autonomic dysfunction and quality‐of‐life questionnaires were also completed. The prevalence of POTS was higher in women with G‐HSD than hEDS and control groups during HUT (43% vs. 7% and 7%, respectively, p < 0.05), but similar between groups during the active stand (47%, 27%, and 13% for G‐HSD, hEDS, and control, respectively). No participants had OH. hEDS and G‐HSD participants reported more severe orthostatic symptoms and poorer quality of life than controls. Although POTS was observed in hypermobile participants, there is no conclusive evidence that its prevalence differed between groups due to differences between the HUT and active stand assessments. Nevertheless, OI and broader autonomic dysfunction impacted on their quality of life. John Wiley & Sons, Inc. 2022-02-27 2022-06 /pmc/articles/PMC9305471/ /pubmed/35224842 http://dx.doi.org/10.1002/ajmg.a.62705 Text en © 2022 The Authors. American Journal of Medical Genetics Part A published by Wiley Periodicals LLC. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ (https://creativecommons.org/licenses/by-nc-nd/4.0/) License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.
spellingShingle Original Articles
Peebles, Karen C.
Tan, Isabella
Butlin, Mark
Collins, Felicity
Tofts, Louise
Avolio, Alberto P.
Pacey, Verity
The prevalence and impact of orthostatic intolerance in young women across the hypermobility spectrum
title The prevalence and impact of orthostatic intolerance in young women across the hypermobility spectrum
title_full The prevalence and impact of orthostatic intolerance in young women across the hypermobility spectrum
title_fullStr The prevalence and impact of orthostatic intolerance in young women across the hypermobility spectrum
title_full_unstemmed The prevalence and impact of orthostatic intolerance in young women across the hypermobility spectrum
title_short The prevalence and impact of orthostatic intolerance in young women across the hypermobility spectrum
title_sort prevalence and impact of orthostatic intolerance in young women across the hypermobility spectrum
topic Original Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9305471/
https://www.ncbi.nlm.nih.gov/pubmed/35224842
http://dx.doi.org/10.1002/ajmg.a.62705
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