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一名北美印第安人患马凡综合征并伴有冠状动脉病变

Marfan syndrome with coronary artery lesions in a North American Indian.

作者信息

Rabkin S W, Corbett B N, Benediktsson H

出版信息

Can Med Assoc J. 1976 Oct 9;115(7):651-3.

Abstract

Marfan syndrome has not been well documented in North American Indians. A 19-year old Ojibwa man had evidence of this syndrome -- specifically, tall stature, long, thin extremities (particularly, fingers and toes), increased urinary excretion of hydroxyproline, aortic aneurysm, aortic regurgitation and pathologic evidence of aortic rupture and alastic tissue fragmentation. Intimal hyperplasia was present in the extramural coronary arteries, while the intramural arteries, usually thought to be involved, were normal.

摘要

北美印第安人中马方综合征的相关记录并不完善。一名19岁的奥吉布瓦族男性有该综合征的相关证据,具体表现为身材高大、四肢细长(尤其是手指和脚趾)、羟脯氨酸尿排泄增加、主动脉瘤、主动脉瓣反流以及主动脉破裂和弹性组织破碎的病理证据。壁外冠状动脉存在内膜增生,而通常认为会受累的壁内动脉则正常。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cbb7/1878770/69e27c355347/canmedaj01489-0065-a.jpg

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本文引用的文献

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The wrist sign. A useful physical finding in the Marfan syndrome.
Arch Intern Med. 1970 Aug;126(2):276-7. doi: 10.1001/archinte.126.2.276.

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