There is a high prevalence of myofascial pain in people with hypermobile Ehlers-Danlos Syndrome (hEDS). The fascial origin of pain may correspond to changes in the extracellular matrix. The objective of this study was to investigate structural changes in fascia in hEDS. A series of 65 patients were examined prospectively-26 with hEDS, and 39 subjects with chronic neck, knee, or back pain without hEDS. The deep fascia of the sternocleidomastoid, iliotibial tract, and iliac fascia were examined with B-mode ultrasound and strain elastography, and the thicknesses were measured. Stiffness (strain index) was measured semi-quantitatively using elastography comparing fascia to muscle. Differences between groups were compared using one-way analysis of variance. hEDS subjects had a higher mean thickness in the deep fascia of the sternocleidomastoid compared with non-hEDS subjects. There was no significant difference in thickness of the iliac fascia and iliotibial tract between groups. Non-hEDS subjects with pain had a higher strain index (more softening of the fascia with relative stiffening of the muscle) compared with hEDS subjects and non-hEDS subjects without back or knee pain. In myofascial pain, softening of the fascia may occur from increase in extracellular matrix content and relative increase in stiffness of the muscle; this change is not as pronounced in hEDS.
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http://dx.doi.org/10.1002/ajmg.c.31948 | DOI Listing |
Cureus
October 2024
Anesthesiology, Unidade Local de Saúde de Braga, Braga, PRT.
Am J Med Genet A
December 2024
Division of Genetic and Genomic Medicine, Center for Cardiovascular Research, Nationwide Children's Hospital, Columbus, Ohio, USA.
Vascular Ehlers-Danlos, Marfan and Loeys-Dietz syndromes have increased risk of aortic dilation and dissection. Previous early studies showed hypermobile Ehlers-Danlos syndrome (hEDS) may also have increased risk, with echocardiography screening recommended; subsequent studies have not confirmed the risk or recommended echocardiography. This pediatric-based study assessed aortic dilation prevalence in those with hEDS by serial echocardiographic examinations and assessed family history for aortic dissections.
View Article and Find Full Text PDFCureus
June 2024
Department of Pediatrics, Ascension St. Vincent's, Jacksonville, USA.
Ehlers-Danlos syndrome (EDS) is a collection of genetic disorders caused by abnormalities in collagen and typified by hyperflexible joints, hyperextensible skin, and a tendency for easy bruising and tissue injuries. Hypermobile Ehlers-Danlos syndrome (hEDS), the most common subtype, presents a diagnostic challenge due to the lack of specific genetic markers. This case report describes a 13-year-old girl with hEDS, presenting with hypermobility, thoracolumbar scoliosis, constipation, glucosuria, microscopic hematuria, urticaria, and intermittent episodes of bilateral hand and feet swelling.
View Article and Find Full Text PDFSci Rep
April 2024
Univ. Lille, Univ. Artois, Univ. Littoral Côte d'Opale, ULR 7369 - URePSSS - Unité de Recherche Pluridisciplinaire Sport Santé Société, 59000, Lille, France.
Breathing difficulties and exertional dyspnea are frequently reported in hypermobile Ehlers-Danlos syndrome (hEDS); however, they are not clearly explained. An impaired proprioception or the addition of a cognitive task could influence ventilatory control. How can the perception of lung volume be measured? Is lung volume perception impaired in hEDS patients? Is the breathing control impaired during a cognitive task in hEDS patients? A device was developed to assess the accuracy of lung volume perception in patients with hEDS and matched control subjects.
View Article and Find Full Text PDFFront Psychol
February 2023
Department of Clinical and Health Psychology, University Autonomous of Barcelona, Barcelona, Spain.
Background: Vascular Ehlers-Danlos (vEDS) and Loeys-Dietz syndromes (LDS) are hereditary disorders of connective tissue having severe vascular complications (HDCTv) which lead to an increased risk of premature death. Little is known about the impact of the disease in patient's daily life.
Method: Sixteen HDCTv patients (vEDS = 9 and LDS = 7), 16 age and sex-matched hypermobile Ehlers-Danlos syndrome patients (hEDS) and 18 healthy subjects (HS), responded to self-questionnaires assessing psychosocial adjustment, quality of life (QoL), anxiety, depression, pain, fatigue and sleep problems.
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