Ehlers-Danlos syndrome type IV, autosomal dominant

Associated with spontaneous rupture of bowel or large arteries; defect in collagen type 3. Management: 1) regular evaluation witrh ultrasound or computed tomography for early detection of aneurysms 2) surgical repair of aneurysms may be difficult because of increased frability of tissues

Database Correlations

OMIM correlations MORBIDMAP 120180

References

Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 2189-91