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Vascular Ehlers-Danlos Syndrome: Imaging Findings
American Journal of Roentgenology, 2007Vascular Ehlers-Danlos syndrome (EDS), formerly known as EDS type IV, is an autosomal dominant disorder characterized by fragility of medium and large arteries due to type III procollagen deficiency. Our purpose was to review the imaging findings in a cohort of patients with a diagnosis of vascular EDS.The radiologic, surgical, and genetic databases at
Thanila A Macedo +2 more
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Vascular Complications of Ehlers-Danlos Syndrome: CT Findings
American Journal of Roentgenology, 2012Patients with vascular Ehlers-Danlos syndrome are predisposed to premature vascular complications. The objective of this article is to review the CT findings and current thoughts about the management of vascular pathologic abnormalities and diseases in patients with Ehlers-Danlos syndrome.The manifestation of vascular Ehlers-Danlos syndrome frequently ...
Linda C Chu +2 more
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The dysmorphic phenotype in vascular Ehlers Danlos syndrome
Clinical Dysmorphology, 2022The Ehlers Danlos syndromes are identified by their connective tissue features and are not rich in dysmorphic handles. Vascular Ehlers Danlos syndrome (vEDS) however, is characterised by a recognisable phenotypic constellation of internal and external dysmorphology.
James R, Lyness, Patrick J, Morrison
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Haemothorax in vascular Ehlers-Danlos syndrome
Reumatología Clínica (English Edition), 2019Vascular Ehlers-Danlos syndrome (EDS IV) is a rare genetic disorder characterized by an alteration in the COL3A1 gene which encodes type III collagen. It is the most common type of collagen in vessels of medium size and certain organs such as the intestines and the uterus.
Kevin, Álvarez +2 more
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The vascular ehlers-danlos syndrome
Current Treatment Options in Cardiovascular Medicine, 2006Vascular Ehlers-Danlos syndrome (EDS) is a life-threatening inherited disorder of connective tissue causing severe arterial and gastrointestinal fragility and rupture, as well as complications of surgical and radiologic interventions. The diagnosis should be considered in patients under the age of 45 years who present with arterial tearing or ...
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Vascular aspects of the Ehlers-Danlos Syndromes
Matrix Biology, 2018The Ehlers-Danlos Syndromes comprise a heterogeneous group of rare monogenic conditions that are characterized by joint hypermobility, skin and vascular fragility and generalized connective tissue friability. The latest classification recognizes 13 clinical subtypes, with mutations identified in 19 different genes.
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Vascular Manifestations in Patients With Ehlers-Danlos Syndrome
Archives of Surgery, 1982Ehlers-Danlos syndrome (EDS) is clinically and genetically a heterogenous disorder of connective tissue synthesis. Seven clinical types of this disease have been identified and the underlying biochemical defects defined in types IV through VII. Unfortunately, most patients with major vascular complications of EDS have few, if any of the commonly ...
G C, Hunter +4 more
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Contemporary management of vascular Ehlers–Danlos syndrome
Current Opinion in Cardiology, 2011Vascular Ehlers-Danlos syndrome (EDS) results from mutations in the formation of type III collagen. This leads to various potentially lethal complications including rupture of the arterial vessels, intestinal organs, and the uterus. This review summarizes recent cohort studies that have improved our medical and surgical management of complications ...
Ying Wei, Lum +2 more
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Treatment of Vascular Ehlers-Danlos Syndrome
Annals of Surgery, 2013To provide the collected evidence from all literature reports.Vascular Ehlers-Danlos syndrome (EDS) is a rare connective tissue disorder with serious hemorrhagic consequences. Most experience on treatment is based on case reports and small case series.A systematic literature review was performed.
David, Bergqvist +2 more
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Successful multidisciplinary management of vascular Ehlers–Danlos syndrome
Clinical Journal of Gastroenterology, 2021Vascular Ehlers-Danlos syndrome is a rare connective tissue disease with a high risk of severe complications. Because of these complications, the median life expectancy for patients with vascular Ehlers-Danlos syndrome is estimated at 48 years. However, the optimal management of these complications remains unclear. A 25-year-old man with abdominal pain
Shintaro Kanaka +7 more
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