Sunday, March 31, 2019

Ehlers Danlos type 4- vascular type VED

features

Col3a1 gene codes for type 3 procollagen
autosomal dominant
arterial dissection and rupture
risk of SMT
thin translucent skin
typical facies
porencephaly

may NOT have hyperelastic skin
diagnose with death during childbirth, hemorrhage after minor trauma or surgery, bowel rupture
median survival 51
Aneurysms are common in VED but VED is not common in aneurysms. No 1 vessel is ICA
Complications of catheterization as high as 67 percent with 17 percent mortality
CCF are common as are dissections of many vessels

No comments: