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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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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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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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Two vascular complications of the Ehlers—Danlos syndrome
European Journal of Vascular Surgery, 1993Patients with Ehlers-Danlos syndrome type IV have thin walled, friable arteries and veins and are prone to spontaneous arterial rupture. We present a patient who suffered severe damage to the common femoral vein during surgery for varicose veins and who subsequently suffered a spontaneous rupture of the right external carotid artery, treated by ...
S, Brearley, J, Fowler, J D, Hamer
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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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Current management of the vascular subtype of Ehlers–Danlos syndrome
Current Opinion in Cardiology, 2020Purpose of review Vascular Ehlers–Danlos syndrome (vEDS) is the most severe form of EDS, affecting the synthesis of type III collagen. It is notable for decreased life expectancy and morbidity, including spontaneous vessel rupture.
Ehsan, Benrashid, John Westley, Ohman
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Stroke in vascular Ehlers-Danlos syndrome
Practical Neurology, 2023Rebecca Ranzani Martins +7 more
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[Vascular Ehlers-Danlos syndrome].
Presse medicale (Paris, France : 1983), 2007The vascular type of Ehlers-Danlos syndrome (EDS) is a rare genetic disease transmitted as an autosomal dominant trait. It is distinguished from other forms of EDS by its unstable acrogeric morphotype and by vascular, gastrointestinal, and obstetrical complications.
Jérôme, Perdu +15 more
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Changing Patterns in the Vascular Form of Ehlers-Danlos Syndrome
Archives of Surgery, 1986We report and analyze two cases of Ehlers-Danlos syndrome (EDS) type 4. The first manifestation of the disease was a spontaneous perforation of the colon in a 47-year-old man; he was successfully reoperated on five years later for the rupture of an abdominal aortic aneurysm.
P, Gertsch +3 more
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Understanding vascular Ehlers-Danlos syndrome
Impact, 2018The discovery of experimentally derived induced pluripotent stem cells (iPSCs) has fostered prospects of patient-specific cell replacement therapies, novel toxicology and drug screening assays, and informative cell models for understanding disease pathogenesis.
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