scholarly journals Association between Postural Orthostatic Tachycardia Syndrome and Joint Hypermobility

2021 ◽  
Vol 2021 ◽  
pp. 1-4
Author(s):  
Alexis Javier Atuesta-Rodriguez ◽  
Yimy F. Medina-Velasquez ◽  
Orfa Motta ◽  
Maria Isabel Narvaez-Medina ◽  
Federico Rondon-Herrera

Joint hypermobility syndrome refers to increased joint flexibility beyond the normal range of motion. This syndrome has a benign form known as Ehlers-Danlos syndrome type 3. This is a disorder in which hypermobility is accompanied by clinical manifestations in the absence of any systemic disease. A clinical finding associated with this condition is postural orthostatic tachycardia syndrome. The following is a rare case of joint hypermobility syndrome and postural orthostatic tachycardia syndrome. The relevance of this case report lies in the impact that this disease had on the patient’s quality of life and the limitation in the performance of activities of daily living.

2013 ◽  
Vol 2013 ◽  
pp. 1-7 ◽  
Author(s):  
Claudia Celletti ◽  
Marco Castori ◽  
Giuseppe La Torre ◽  
Filippo Camerota

Ehlers-Danlos syndrome hypermobility type a. k. a. joint hypermobility syndrome (JHS/EDS-HT) is a hereditary musculoskeletal disorder associating generalized joint hypermobility with chronic pain. Anecdotal reports suggest a prominent role forkinesiophobiain disease manifestations, but no study has systematically addressed this point.Objective. To investigate the impact ofkinesiophobiaand its relationship with pain, fatigue, and quality of life in JHS/EDS-HT.Design. Cross-sectional study.Subjects/Patients. 42 patients (40 female and 2 male) with JHS/EDS-HT diagnosis following standardized diagnostic criteria were selected.Methods. Disease features were analyzed by means of specific questionnaires and scales evaluating kinesiophobia, pain, fatigue, and quality of life. The relationships among variables were investigated using the Spearman bivariate analysis.Results.Kinesiophobiaresulted predominantly in the patients’ sample. The values ofkinesiophobiadid not correlate with intensity of pain, quality of life, and (or) the single component of fatigue. A strong correlation was discovered betweenkinesiophobiaand general severity of fatigue.Conclusions. In JHS/EDS-HT, the onset of pain-avoiding strategies is related to the presence of pain but not to its intensity. The clear-cut correlation betweenkinesiophobiaand severity of fatigue suggests a direct link between musculoskeletal pain and fatigue. In JHS/EDS-HT, the underlying mechanism is likely to be facilitated by primary disease characteristics, including hypotonia.


2017 ◽  
Vol 2017 ◽  
pp. 1-7 ◽  
Author(s):  
Claudia Celletti ◽  
Filippo Camerota ◽  
Marco Castori ◽  
Federica Censi ◽  
Laura Gioffrè ◽  
...  

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.


2014 ◽  
Vol 28 (11) ◽  
pp. 4668-4676 ◽  
Author(s):  
Rie Harboe Nielsen ◽  
Christian Couppé ◽  
Jacob Kildevang Jensen ◽  
Morten Raun Olsen ◽  
Katja Maria Heinemeier ◽  
...  

2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
Trinh Hermanns-Lê ◽  
Marie-Annick Reginster ◽  
Claudine Piérard-Franchimont ◽  
Philippe Delvenne ◽  
Gérald E. Piérard ◽  
...  

The distinction between the Ehlers-Danlos syndrome hypermobile type (EDSH) and the benign joint hypermobility syndrome (BJHS) is unclear. The aim of the present study was to compare skin ultrastructural abnormalities of EDSH and BJHS among different families. Skin of 23 EDSH, 27 BJHS, and 41 asymptomatic subjects from 17 families was examined using transmission electron microscopy. Similar ultrastructural abnormalities were found irrespective of the Beighton score. Flower-like collagen fibrils represented the key change and elastic fibers were altered as well. Beighton score is a clinical parameter rating joint mobility that appeared unrelated to quantitative and qualitative collagen ultrastructural alterations in the skin. Some EDSH family members fit with BJHS diagnosis. BJHS possibly represents a mild variant of EDSH.


MYOPAIN ◽  
2015 ◽  
Vol 23 (1-2) ◽  
pp. 28-33 ◽  
Author(s):  
Ebru Sahin ◽  
Selmin Gulbahar ◽  
Meltem Baydar ◽  
Gozde Ozcan Soylev ◽  
Cigdem Bircan ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document