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Orthostatic Intolerance and Postural Orthostatic Tachycardia Syndrome in Joint Hypermobility Syndrome/Ehlers-Danlos Syndrome, Hypermobility Type: Neurovegetative Dysregulation or Autonomic Failure?

Abstract Background. Joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type (JHS/EDS-HT), is a hereditary connective tissue disorder mainly characterized by generalized joint hypermobility, skin texture abnormalities, and visceral and vascular dysfunctions, also comprising symptoms of autonomic dysfunction. This study aims to further evaluate cardiovascular autonomic involvement in JHS/EDS-HT by a battery of functional tests. Methods. The response to cardiovascular reflex tests comprising deep breathing, Valsalva maneuver, 30/15 ratio, handgrip test, and head-up tilt test was studied in 35 JHS/EDS-HT adults. Heart rate and blood pressure variability was also investigated by spectral analysis in comparison to age and sex healthy matched group. Results. Valsalva ratio was normal in all patients, but 37.2% of them were not able to finish the test. At tilt, 48.6% patients showed postural orthostatic tachycardia, 31.4% orthostatic intolerance, 20% normal results. Only one patient had orthostatic hypotension. Spectral analysis showed significant higher baroreflex sensitivity values at rest compared to controls. Conclusions. This study confirms the abnormal cardiovascular autonomic profile in adults with JHS/EDS-HT and found the higher baroreflex sensitivity as a potential disease marker and clue for future research.
PMID
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Authors

Mayor MeshTerms

Baroreflex

Hand Strength

Keywords
Journal Title biomed research international
Publication Year Start




PMID- 28286774
OWN - NLM
STAT- MEDLINE
DA  - 20170313
DCOM- 20170317
LR  - 20170317
IS  - 2314-6141 (Electronic)
VI  - 2017
DP  - 2017
TI  - Orthostatic Intolerance and Postural Orthostatic Tachycardia Syndrome in Joint
      Hypermobility Syndrome/Ehlers-Danlos Syndrome, Hypermobility Type:
      Neurovegetative Dysregulation or Autonomic Failure?
PG  - 9161865
LID - 10.1155/2017/9161865 [doi]
AB  - Background. Joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility
      type (JHS/EDS-HT), is a hereditary connective tissue disorder mainly
      characterized by generalized joint hypermobility, skin texture abnormalities, and
      visceral and vascular dysfunctions, also comprising symptoms of autonomic
      dysfunction. This study aims to further evaluate cardiovascular autonomic
      involvement in JHS/EDS-HT by a battery of functional tests. Methods. The response
      to cardiovascular reflex tests comprising deep breathing, Valsalva maneuver,
      30/15 ratio, handgrip test, and head-up tilt test was studied in 35 JHS/EDS-HT
      adults. Heart rate and blood pressure variability was also investigated by
      spectral analysis in comparison to age and sex healthy matched group. Results.
      Valsalva ratio was normal in all patients, but 37.2% of them were not able to
      finish the test. At tilt, 48.6% patients showed postural orthostatic tachycardia,
      31.4% orthostatic intolerance, 20% normal results. Only one patient had
      orthostatic hypotension. Spectral analysis showed significant higher baroreflex
      sensitivity values at rest compared to controls. Conclusions. This study confirms
      the abnormal cardiovascular autonomic profile in adults with JHS/EDS-HT and found
      the higher baroreflex sensitivity as a potential disease marker and clue for
      future research.
FAU - Celletti, Claudia
AU  - Celletti C
AUID- ORCID: 0000-0001-7154-5882
AD  - Physical Medicine and Rehabilitation, Umberto I Hospital, Rome, Italy.
FAU - Camerota, Filippo
AU  - Camerota F
AUID- ORCID: 0000-0002-0268-4026
AD  - Physical Medicine and Rehabilitation, Umberto I Hospital, Rome, Italy.
FAU - Castori, Marco
AU  - Castori M
AD  - Unit of Clinical Genetics, San Camillo-Forlanini Hospital, Rome, Italy.
FAU - Censi, Federica
AU  - Censi F
AD  - Department of Cardiovascular, Dysmetabolic and Aging-Associated Diseases, Italian
      Institute of Health, Rome, Italy.
FAU - Gioffre, Laura
AU  - Gioffre L
AD  - Department of Heart and Great Vessels "A. Reale", Sapienza University of Rome,
      Rome, Italy.
FAU - Calcagnini, Giovanni
AU  - Calcagnini G
AD  - Department of Cardiovascular, Dysmetabolic and Aging-Associated Diseases, Italian
      Institute of Health, Rome, Italy.
FAU - Strano, Stefano
AU  - Strano S
AD  - Department of Heart and Great Vessels "A. Reale", Sapienza University of Rome,
      Rome, Italy.
LA  - eng
PT  - Clinical Trial
PT  - Journal Article
DEP - 20170212
PL  - United States
TA  - Biomed Res Int
JT  - BioMed research international
JID - 101600173
SB  - IM
MH  - Adult
MH  - Autonomic Nervous System Diseases/*physiopathology
MH  - *Baroreflex
MH  - Ehlers-Danlos Syndrome/*physiopathology
MH  - Female
MH  - *Hand Strength
MH  - Humans
MH  - Joint Instability/*physiopathology
MH  - Male
MH  - Middle Aged
MH  - Neurodegenerative Diseases/*physiopathology
MH  - Postural Orthostatic Tachycardia Syndrome/*physiopathology
PMC - PMC5329674
COI - The authors declare that there is no conflict of interests regarding the
      publication of this paper.
EDAT- 2017/03/14 06:00
MHDA- 2017/03/18 06:00
CRDT- 2017/03/14 06:00
PHST- 2016/10/31 [received]
PHST- 2017/01/16 [revised]
PHST- 2017/01/19 [accepted]
AID - 10.1155/2017/9161865 [doi]
PST - ppublish
SO  - Biomed Res Int. 2017;2017:9161865. doi: 10.1155/2017/9161865. Epub 2017 Feb 12.

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