다발뇌혈관박리로 발현한 혈관엘러스-단로스증후군의 예
A Case of Vascular Ehlers-Danlos Syndrome Presenting with Multiple Cerebral Arterial Dissections
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초록
Vascular EhlersDanlos syndrome (vEDS) is caused by dysfunctional COL3A1 gene for type III procollagen. It presents with translucent skin, easy bruising, and arterial dissection. It has the worst prognosis among various types of EDS with holloworgan rupture as the leading cause of death, mounting to the median life expectancy of mid40s. We report a rare case of vascular EhlersDanlos syndrome presenting late in the fifth decade of life with multiple arterial dissections isolated to cerebral arteries.
Abstract
Vascular EhlersDanlos syndrome (vEDS) is caused by dysfunctional COL3A1 gene for type III procollagen. It presents with translucent skin, easy bruising, and arterial dissection. It has the worst prognosis among various types of EDS with holloworgan rupture as the leading cause of death, mounting to the median life expectancy of mid40s. We report a rare case of vascular EhlersDanlos syndrome presenting late in the fifth decade of life with multiple arterial dissections isolated to cerebral arteries.
참고문헌 (10)
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