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J R Soc Med. 1995 September; 88(9): 532P–533P.
PMCID: PMC1295338

Renal autotransplantation for aortic dissection in Marfan's syndrome.

Abstract

Marfan's syndrome is an autosomal dominant condition with a prevalence of 4 to 6:100,000, and classically comprises skeletal changes, ectopia lentis and cystic medial necrosis of the largest arteries. The latter leads to aneurysm formation, most often commencing at the aortic root, and is responsible for an average age at death of 32 years. We describe successful preservation of threatened renal function by renal autotransplantation, without concurrent aortic replacement, following dissection of the descending aorta in a patient with Marfan's syndrome.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.
  • Pyeritz RE, McKusick VA. The Marfan syndrome: diagnosis and management. N Engl J Med. 1979 Apr 5;300(14):772–777. [PubMed]
  • Murdoch JL, Walker BA, Halpern BL, Kuzma JW, McKusick VA. Life expectancy and causes of death in the Marfan syndrome. N Engl J Med. 1972 Apr 13;286(15):804–808. [PubMed]
  • Crawford ES, Coselli JS, Svensson LG, Safi HJ, Hess KR. Diffuse aneurysmal disease (chronic aortic dissection, Marfan, and mega aorta syndromes) and multiple aneurysm. Treatment by subtotal and total aortic replacement emphasizing the elephant trunk operation. Ann Surg. 1990 May;211(5):521–537. [PubMed]
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