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Vascular Ehlers–Danlos syndrome (vEDS) is a rare connective tissue disorder due to heterozygous mutations in the COL3A1 gene with a dominant negative effect. Spontaneous bowel perforation and intra‐abdominal organ rupture… Click to show full abstract
Vascular Ehlers–Danlos syndrome (vEDS) is a rare connective tissue disorder due to heterozygous mutations in the COL3A1 gene with a dominant negative effect. Spontaneous bowel perforation and intra‐abdominal organ rupture are common complications of vEDS. Other gastrointestinal (GI) manifestations may occur but have not been extensively characterized. We herein describe the natural history of GI events and surgery‐related complications in patients with vEDS.
Keywords:
gastrointestinal manifestations;
danlos syndrome;
vascular ehlers;
natural history;
ehlers danlos
Journal Title: Journal of Gastroenterology and Hepatology
Year Published: 2019
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Journal Title: Journal of Gastroenterology and Hepatology
Year Published: 2019
Link to full text (if available)
Share on Social Media: Sign Up to like & get
recommendations!