엘러스-단로스 증후군에 발생한 자발성 신장동맥 박리
Spontaneous renal artery dissection in Ehlers-Danlos syndrome
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초록
Primary dissection of the renal artery is rare. Spontaneous renal artery dissection can be associated with diseases such as medial degeneration, neurofibromatosis, syphilitic arteritis, tuberculosis, polyarteritis nodosa, Marfan syndrome, fibromuscular dysplasia, or Ehlers-Danlos syndrome (EDS). Among these causes, EDS related renal artery dissection is very rare worldwide and has not been previously reported in Korea. EDS are a group of heritable connective tissue disorders characterized by fragility of the skin and hypermobility of the joints. We describe the case history of a young man who presented with left side flank pain, hypermobility of the hand joints and showed left renal artery dissection on computed tomography and angiography that turned out to be the first complication of vascular type EDS.
Abstract
Primary dissection of the renal artery is rare. Spontaneous renal artery dissection can be associated with diseases such as medial degeneration, neurofibromatosis, syphilitic arteritis, tuberculosis, polyarteritis nodosa, Marfan syndrome, fibromuscular dysplasia, or Ehlers-Danlos syndrome (EDS). Among these causes, EDS related renal artery dissection is very rare worldwide and has not been previously reported in Korea. EDS are a group of heritable connective tissue disorders characterized by fragility of the skin and hypermobility of the joints. We describe the case history of a young man who presented with left side flank pain, hypermobility of the hand joints and showed left renal artery dissection on computed tomography and angiography that turned out to be the first complication of vascular type EDS.
참고문헌 (13)
- Wooley CF, "Functional abnormalities of the aorta", pp.295~320 (1996), Futura Publishing
- Esayag-Tendler B, "Accelerated hypertension with encephalopathy due to an isolated dissection of a renal artery branch vessel", Am J Kidney Dis, 23, pp.869~873 (1994)
- Beighton P, "Ehlers-Danlos syndromes: revised nosology, Villefranche, 1997. Ehlers-Danlos National Foundation (USA) and Ehlers-Danlos Support Group (UK)", Am J Med Genet, 77, pp.31~37 (1998)
- Germain DP., "Ehlers-Danlos syndrome type IV", Orphanet J Rare Dis, 2, pp.32~ (2007)
- Pepin M, "Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type", N Engl J Med, 342, pp.673~680 (2000)
- Zilocchi M, "Vascular Ehlers-Danlos syndrome: imaging findings", AJR Am J Roentgenol, 189, pp.712~719 (2007)
- 박종권, "제4형 엘러스-단로스 증후군에 속발된 혈관 합병증의 치료", Vascular Specialist International, 27(2), pp.47~51 (2011), 대한혈관외과학회
- Oderich GS, "The spectrum, management and clinical outcome of Ehlers-Danlos syndrome type IV: a 30-year experience", J Vasc Surg, 42, pp.98~106 (2005)
- Okada T, "Embolization of life-threatening arterial rupture in patients with vascular Ehlers-Danlos syndrome", Cardiovasc Intervent Radiol, 37, pp.77~84 (2014)
- Casana R, "Endovascular treatment of hepatic artery aneurysm in a patient with Ehlers-Danlos syndrome", Case report. Int Angiol, 23, pp.291~295 (2004)
- Tonnessen BH, "Endovascular repair of an iliac artery aneurysm in a patient with Ehlers-Danlos syndrome type IV", J Vasc Surg, 45, pp.177~179 (2007)
- Khalique Z, "Successful endovascular repair of acute type B aortic dissection in undiagnosed Ehlers-Danlos syndrome type IV", Eur J Vasc Endovasc Surg, 38, pp.608~609 (2009)
- Raval M, "Covered stent use after subclavian artery and vein injuries in the setting of vascular Ehlers-Danlos", J Vasc Surg, 55, pp.542~544 (2012)