Lethal Complications of the Ehlers-Danlos Syndrome
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چکیده
منابع مشابه
Lethal complications of the Ehlers-Danlos syndrome.
Death from arterial bleeding or gastrointestinal perforation has previously been reported in more than 15 patients with the Ehlers-Danlos syndrome. On a basis of 100 personally examined patients and a review of published accounts, these events seem particularly likely to occur in two distinct and recognizable forms (the gravis and the ecchymotic forms) of the syndrome.
متن کاملGastrointestinal complications of the Ehlers-Danlos syndrome.
The gastrointestinal abnormalities encountered in 125 patients with the Ehlers-Danlos syndrome have been described. Spontaneous perforation of the intestine and massive gastrointestinal haemorrhage are uncommon but potentially lethal complications of the Ehlers-Danlos syndrome. Less dangerous abnormalities, such ;as external hernia, hiatus hernia, eventration of the diaphragm, intestinal divert...
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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.
متن کاملSerious ophthalmological complications in the Ehlers-Danlos syndrome.
The main features of the Ehlers-Danlos syndrome (EDS) are cutaneous hyperextensibility, articular laxity, and fragility of the tissues. Systemic ramifications may lead to orthopaedic, cardiovascular, and gastrointestinal problems. Minor ophthalmological changes, including epicanthic folds, strabismus, and myopia are not infrequent, but serious ocular complications are rare. However, there have ...
متن کاملEhlers-Danlos syndrome: complications and solutions concerning anesthetic management.
Ehlers-Danlos syndrome is an inherited disorder that results in dysfunctional collagen bundles. These dysfunctional collagen bundles are most noticeable in tissues rich with collagen fibers--skin, vessels, GI, and ligaments. Until gene therapy advancements can correct the underlying gene mutations causing faulty collagen, the mainstay of treatment is prevention of traumatic injury. The success ...
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ژورنال
عنوان ژورنال: BMJ
سال: 1968
ISSN: 0959-8138,1468-5833
DOI: 10.1136/bmj.3.5619.656