CORONARY ARTERY DISSECTION IN EHLERS DANLOS SYNDROME
نویسندگان
چکیده
منابع مشابه
[Ehlers-Danlos syndrome].
We have described a patient with type 4 Ehlers-Danlos syndrome who died of a ruptured pulmonary artery. Of the six subtypes of Ehlers-Danlos syndrome, which can usually be clinically differentiated, only types 1 and 4 appear to be associated with a substantial risk of arterial rupture.
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Background: Ehlers-Danlos syndrome type IV -vascular typeis an inherited connective tissue disorder characterized by several clinical features: easy bruisability, translucent skin, acrogeria and uterine, intestinal or arterial rupture. The arteries most frequently involved are the iliac, splenic, renal arteries and the aorta. Diagnosis is made utilizing clinical criteria (Beighton) and collagen...
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Ehlers-Danlos syndrome (EDS) is a rare heterogeneous group of connective tissue disorders. The vascular type (vEDS) is an autosomal dominant disorder caused by heterozygous mutations in the COL3A1 gene predisposing to premature arterial, intestinal, or uterine rupture. We report a case of a 38-year-old woman with a recent diagnosis of vEDS admitted in the Emergency Department with a suspicion o...
متن کاملEhlers-Danlos syndrome.
Twenty patients with the Ehlers-Danlos syndrome, (10 type I, six type II, and four type IV) were studied to assess the frequency of respiratory abnormalities in this condition. Five patients (25%) had had at least one episode of haemoptysis, but none had any defect of coagulation. There was a high frequency of recurrent sinusitis, notably in those with the type I syndrome. Two patients had bull...
متن کاملEhlers-Danlos syndrome.
448 BMJ | 1 SEPTEMBER 2007 | VoluME 335 hinder implementation of these recommendations. Recognising the condition and the impact it can have on the lives of patients and their families is therefore a necessary preliminary to improving care. • People with severe CFS/ME who are housebound or need prolonged bed rest require specialised care, but evidence is lacking about which treatments are most ...
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ژورنال
عنوان ژورنال: Journal of the American College of Cardiology
سال: 2016
ISSN: 0735-1097
DOI: 10.1016/s0735-1097(16)31019-1