Korean J Med.
2022 Aug;97(4):271-276. 10.3904/kjm.2022.97.4.271.
Renal Infarction in Vascular Ehlers-Danlos Syndrome
- Affiliations
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- 1Divison of Nephrology, Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
- 2Division of Nephrology, Department of Internal Medicine, Dankook University Hospital, Dankook University College of Medicine, Cheonan, Korea
- KMID: 2532483
- DOI: http://doi.org/10.3904/kjm.2022.97.4.271
Abstract
- Renal infarction is a condition caused by sudden disturbance of renal arterial blood flow, which occurs frequently in cardioembolic disease, renal artery injury (trauma, Marfan syndrome, rarely Ehlers-Danlos syndrome), and in association with a hypercoagulable state. Ehlers-Danlos syndrome is a rare hereditary connective tissue disease characterized by skin fragility, hyperelasticity, hypermobility of small joints, and easy bruising. Among the subtypes, vascular Ehlers-Danlos syndrome is a very rare genetic disease caused by a mutation in the COL3A1 gene. As blood vessels are fragile due dysfunctional collagen synthesis, complications of bleeding due to arterial dissection or rupture are common in patients with this disease. Here, we report a case of vascular Ehlers-Danlos syndrome diagnosed based on a renal infarction caused by thrombus and renal artery injury; we also present a review of the relevant literature.
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