Subacromial space outlet in female patients with multidirectional instability based on hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorder measured by ultrasound

The objective of the study was to compare the acromiohumeral distance (AHD) between patients diagnosed with hypermobility type of the Ehlers-Danlos syndrome (hEDS) or hypermobility spectrum disorder (HSD) and healthy controls by evaluating the relative amount the tendon occupies in the subacromial area. Furthermore, the aim was to evaluate if there was a change in AHD with arm elevation within and between groups.
Source: Journal of Shoulder and Elbow Surgery - Category: Orthopaedics Authors: Source Type: research

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Guillaume Goudot1, Tristan Mirault2,3, Patrick Bruneval2,4, Gilles Soulat5, Mathieu Pernot1 and Emmanuel Messas2,3* 1INSERM U1273, ESPCI Paris, CNRS FRE 2031, Physics for Medicine Paris, PSL Research University, Paris, France 2Centre de Référence des Maladies Vasculaires Rares, Hôpital Européen Georges-Pompidou, Assistance Publique – Hôpitaux de Paris (APHP), Paris, France 3INSERM U970 PARCC, Paris Descartes University – Sorbonne Paris Cité University, Paris, France 4Service d’Anatomie Pathologique, Hôpital Européen Georges-Pompidou, Assistanc...
Source: Frontiers in Physiology - Category: Physiology Source Type: research
Arterial Stiffening with Ultrafast Ultrasound Imaging Gives New Insight into Arterial Phenotype of Vascular Ehlers-Danlos Mouse Models. Ultraschall Med. 2018 Sep 21;: Authors: Goudot G, Papadacci C, Dizier B, Baudrie V, Ferreira I, Boisson-Vidal C, Tanter M, Jeunemaître X, Pernot M, Messas E, Mirault T Abstract OBJECTIVE:  Vascular Ehlers-Danlos syndrome (vEDS) is associated with arterial ruptures due to a mutant gene encoding collagen type III (Col-III). To better understand the role of Col-III, we aimed at evaluating aortic stiffness and dynamic stiffening in vEDS mouse models, ...
Source: Ultraschall in der Medizin - Category: Radiology Authors: Tags: Ultraschall Med Source Type: research
Conditions:   Ehlers-Danlos Syndrome;   Fibromuscular Dysplasia Intervention:   Device: Ultra High Frequency ultrasound system Sponsors:   Assistance Publique - Hôpitaux de Paris;   Institut National de la Santé Et de la Recherche Médicale, France Not yet recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
Authors: Vandamme M, De Backer J, De Backer T, Drieghe B, Devos D, Gevaert S Abstract Spontaneous coronary artery dissection or SCAD is a rare and challenging disease that is increasingly diagnosed. It is characterized by a non-traumatic, non-iatrogenic separation of the coronary artery wall and occurs predominantly in young and middle-aged women without traditional cardiovascular risk factors. SCAD is often associated with predisposing conditions such as the peripartum period, systemic inflammatory disease and heritable connective tissue disease. More recently, independent investigators have demonstrated an import...
Source: Acta Cardiologica - Category: Cardiology Tags: Acta Cardiol Source Type: research
Vascular Ehlers Danlos syndrome (vEDS) is an uncommon genetic disorders characterized by arterial aneurysm, dissection and rupture, bowel rupture, and rupture of the gravid uterus. The frequency is estimated as 1/50,000–1/200,000 and results from pathogenic variants in COL3A1, which encodes the chains of type III procollagen, a major protein in vessel walls and hollow organs. Initial diagnosis depends on the recognitions of clinical features, including family history. Management is complex and requires multiple specialists who can respond to and manage the major complications. A summary of recommendations for managem...
Source: American Journal of Medical Genetics Part C: Seminars in Medical Genetics - Category: Genetics & Stem Cells Authors: Tags: Research Article Source Type: research
Vascular Ehlers–Danlos Syndrome (vEDS) is caused by heterozygous mutations in COL3A1 and is characterized by fragile vasculature and hollow organs, with a high risk of catastrophic events at a young age. During pregnancy and delivery, maternal mortality rates up until 25% have been reported. However, recent pedigree analysis reported a substantial lower pregnancy‐related mortality rate of 4.9%. Here, we describe an extended vEDS family with multiple uneventful pregnancy outcomes. In the proband, a 37‐year‐old woman, DNA‐analysis because of an asymptomatic iliac artery dissection revealed a pathogenic mutation...
Source: American Journal of Medical Genetics Part A - Category: Genetics & Stem Cells Authors: Tags: Clinical Report Source Type: research
Resumo A Síndrome de Ehlers-Danlos (EDS) é uma rara doença hereditária do tecido conjuntivo proveniente de uma alteração da síntese do colágeno. A principal característica da EDS é a extrema fragilidade do tecido conjuntivo, que pode resultar em rotura uterina, perfuração intestinal espontânea e várias doenças vasculares como aneurismas, pseudoaneurismas, dissecções arteriais e roturas espontâneas. Os autores relatam o caso de um paciente de 11 anos de idade que apresentava tumor pulsátil na fo...
Source: Jornal Vascular Brasileiro - Category: Surgery Source Type: research
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