Ehlers-Danlos syndrome type IV, autosomal dominant
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Introduction
Associated with spontaneous rupture of bowel or large arteries; defect in collagen type 3.
Management
- regular evaluation witrh ultrasound or computed tomography for early detection of aneurysms
- surgical repair of aneurysms may be difficult because of increased frability of tissues
More general terms
Additional terms
References
- ↑ Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 2189-91