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Physiological Reports
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Physiological Reports
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Baroreflex sensitivity in facioscapulohumeral muscular dystrophy

Authors: Miguel Anselmo; Shandon Coffman; Mia Larson; Kathryn Vera; Emma Lee; Mary McConville; Michael Kyba; +1 Authors

Baroreflex sensitivity in facioscapulohumeral muscular dystrophy

Abstract

Facioscapulohumeral muscular dystrophy (FSHD), a common form of muscular dystrophy, is caused by a genetic mutation that alters DUX4 gene expression. This mutation contributes to significant skeletal muscle loss. Although it is suggested that cardiac muscle may be spared, people with FSHD have demonstrated autonomic dysregulation. It is unknown if baroreflex function, an important regulator of blood pressure (BP), is impaired in people with FSHD. We examined if baroreflex sensitivity (BRS) is blunted in patients with FSHD. Thirty minutes of resting BP, heart rate, and cardiovagal BRS were measured in 13 patients with FSHD (age: 50 ± 13 years, avg ± SD) and 17 sex- and age-matched controls (age: 47 ± 14 years, p > 0.05). People with FSHD were less active (Activity Metabolic Index, AMI) (FSHD: 24 ± 30; controls: 222 ± 175 kcal/day; p  0.05). BRSup (hypertensive response), BRSdown (hypotensive response), and total BRS were similar between groups (BRSup: FSHD: 12 ± 8; controls: 12 ± 5 ms/mmHg; BRSdown: FSHD: 10 ± 4; controls: 13 ± 6 ms/mmHg; BRS: FSHD: 14 ± 9; controls: 13 ± 6 ms/mmHg; p > 0.05). Mean arterial pressure was similar between groups (FSHD: 96 ± 7; controls: 91 ± 6mmHg). Individuals with FSHD had an elevated heart rate compared with controls (FSHD: 65 ± 8; controls: 59 ± 8 BPM; p = 0.03), but when co-varied for AMI, this relationship disappeared (p = 0.39). These findings suggest that BRS is not attenuated in people with FSHD, but an elevated heart rate may be due to low physical activity levels, a potential consequence of limited mobility.

Keywords

muscular dystrophy, Adult, Facioscapulohumeral, Physiology, 610, Blood Pressure, Medicine and Health Sciences, Homeobox, QP1-981, Humans, Muscular Dystrophy, Muscle, Skeletal, Homeodomain Proteins, FSHD, heart rate variability, Genes, Homeobox, blood pressure, 600, Skeletal, Original Articles, Baroreflex, Middle Aged, Muscular Dystrophy, Facioscapulohumeral, Rehabilitation and Therapy, Genes, Muscle, autonomic function

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citations
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
0
Average
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