Ehlers-Danlos syndrome type IV

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Ehlers-Danlos syndrome type IV

Ehlers-Danlos syndrome type IV, the vascular type of Ehlers-Danlos syndromes (EDS), is an inherited connective tissue disorder defined by characteristic facial features (acrogeria) in most patients, translucent skin with highly visible subcutaneous vessels on the trunk and lower back, easy bruising, and severe arterial, digestive and uterine complications, which are rarely, if at all, observed ...

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Ehlers-Danlos Syndrome Type VIII

Ehlers-Danlos syndrome (EDS) is a genetically heterogeneous connective tissue disorder which is comprised of more than 10 phenotypes including EDS-VIII (periodontitis type), which is characterized by chronically inflamed pretibial lesions and severe periodontitis. We describe a 26-year-old female with a long-standing history of abnormal scarring tissues, presenting with pretibial waxy violaceou...

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Ehlers-Danlos syndrome (type VIII).

Ehlers-Danlos syndrome (EDS), a group of rare, autosomal dominantly inherited connective tissue dysplasias, characterized mainly by abnormal collagen synthesis, has been shown to exhibit extensive heterogeneity with at least 11 clinical entities differentiated by their clinical, biochemical, and genetic features. Of these, Type VIII EDS is of special interest from a dental viewpoint, due mainly...

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Ehlers-Danlos syndrome, classical type.

Classical EDS is a heritable disorder of connective tissue. Patients are affected with joint hypermobility, skin hyperextensibilty, and skin fragility leading to atrophic scarring and significant bruising. These clinical features suggest consideration of the diagnosis which then needs to be confirmed, preferably by genetic testing. The most recent criteria for the diagnosis of EDS were devised ...

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Ehlers-Danlos syndrome type IV: anesthetic considerations--case report.

This report describes the anesthetic management of a patient with Ehlers-Danlos syndrome type IV. This is one of the rare genetic disorder which can present both in emergency and as a scheduled surgical case.

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ژورنال

عنوان ژورنال: Orphanet Journal of Rare Diseases

سال: 2007

ISSN: 1750-1172

DOI: 10.1186/1750-1172-2-32