|
KMID : 1101620170280040166
|
|
Perinatology
2017 Volume.28 No. 4 p.166 ~ p.170
|
|
|
The Vascular Type Ehlers-Danlos Syndrome Diagnosed after Delivery
|
|
Kim Su-Mi
Kang Yun-Dan
|
|
|
Abstract
|
|
|
|
Vascular Ehlers-Danlos sydrome (vEDS) is a life-threatening autosomal dominant inherited disorder of connective tissue characterized by arterial aneurysm, dissection and rupture, bowel rupture, and rupture of the gravid uterus. vEDS results from mutation of COL3A1, which encodes the chains of type III collagen, a major protein in vessel walls and hollow organs. A 32-year-old primigravida had aneurysmal rupture in right posterior popliteal artery 9 days after induced vaginal delivery. The reason of labor induction was preterm premature rupture of membrane. She was diagnosed with vEDS by sequence analysis of COL3A1 mutation. A multidisciplinary team is required to provide tailored counseling and education about vEDS. Also, a long term follow up is need for individuals with vEDS.
|
|
|
KEYWORD
|
|
|
COL3A1, Ehlers-Danlos syndrome, Type IV, Vascular type, Postpartum, Rupture
|
|
|
FullTexts / Linksout information
|
|
|
|
|
|
Listed journal information
|
|
  
|
|