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马凡综合征的主动脉病变:囊性中层退变的超微结构

Aortic lesion in Marfan syndrome: the ultrastructure of cystic medial degeneration.

作者信息

Saruk M, Eisenstein R

出版信息

Arch Pathol Lab Med. 1977 Feb;101(2):74-7.

PMID:576392
Abstract

Areas of medial alteration (Erdheim cystic medionecrosis) in an aortic root aneurysm surgically removed from a patient with Marfan syndrome were studied with an electron microscope. The single striking morphologic abnormality was the disorganized, haphazard architecture of the media in these areas. This lesion is interpreted as being an abortive reparative response to hemodynamic stress in a condition with a molecular defect in the structural proteins of connective tissue.

摘要

对一名患有马凡综合征的患者手术切除的主动脉根部动脉瘤中内侧改变区域(厄尔德海姆囊性中膜坏死)进行了电子显微镜研究。唯一显著的形态学异常是这些区域中膜结构紊乱、杂乱无章。这种病变被解释为在结缔组织结构蛋白存在分子缺陷的情况下,对血流动力学应激的一种失败的修复反应。

相似文献

1
Aortic lesion in Marfan syndrome: the ultrastructure of cystic medial degeneration.马凡综合征的主动脉病变:囊性中层退变的超微结构
Arch Pathol Lab Med. 1977 Feb;101(2):74-7.
2
Aortic aneurysm in Marfan's syndrome: changes in the ultrastructure and composition of collagen.马凡综合征中的主动脉瘤:胶原蛋白超微结构和组成的变化。
J Anat. 1979 Oct;129(Pt 3):645-57.
3
[Autopsy case of Marfan's syndrome associated with giant aneurysm of the aorta].
Naika. 1968 Dec;22(7):1526-33.
4
[Sudden death in children and adolescents, carriers of an unrecognized cystic medionecrosis of the aorta].
Schweiz Med Wochenschr. 1981 Nov 28;111(48):1829-33.
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Surgery of proximal aorta in Marfan's syndrome.
J Thorac Cardiovasc Surg. 1973 Jul;66(1):75-81.
6
Marfan's syndrome: structural, biochemical, and mechanical studies of the aortic media.马方综合征:主动脉中层的结构、生化及力学研究
J Lab Clin Med. 1985 Oct;106(4):376-83.
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The prevalence of obstructive sleep apnoea and its association with aortic dilatation in Marfan's syndrome.马凡综合征中阻塞性睡眠呼吸暂停的患病率及其与主动脉扩张的关联。
Thorax. 2009 Feb;64(2):162-6. doi: 10.1136/thx.2008.102756. Epub 2008 Oct 13.
8
Variation in the histopathological features of patients with ascending aortic aneurysms: a study of 111 surgically excised cases.升主动脉瘤患者组织病理学特征的变异:111例手术切除病例的研究
J Clin Pathol. 2008 Apr;61(4):519-23. doi: 10.1136/jcp.2006.046250. Epub 2007 Oct 15.
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Histopathologic findings in ascending aortas from individuals with Loeys-Dietz syndrome (LDS).洛伊斯-迪茨综合征(LDS)患者升主动脉的组织病理学发现。
Am J Surg Pathol. 2009 Feb;33(2):194-201. doi: 10.1097/PAS.0b013e31817f3661.
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[Case of sudden death in Marfan's syndrome].[马方综合征猝死病例]
Sud Med Ekspert. 1976 Jul-Sep;19(3):51-2.

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Annulo-aortic ectasia. Light and electron microscopic changes in aortic media.瓣环-主动脉扩张。主动脉中膜的光镜和电镜变化。
Virchows Arch A Pathol Anat Histopathol. 1985;407(3):279-88. doi: 10.1007/BF00710653.
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Unilateral microfibrillar abnormalities in a case of asymmetric Marfan syndrome.不对称性马方综合征病例中的单侧微纤维异常
Am J Hum Genet. 1990 Apr;46(4):661-71.
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Cosegregation of elastin-associated microfibrillar abnormalities with the Marfan phenotype in families.在家族中,弹性蛋白相关微原纤维异常与马凡氏综合征表型的共分离现象。
Am J Hum Genet. 1990 Apr;46(4):652-60.
7
Aortic aneurysm in Marfan's syndrome: changes in the ultrastructure and composition of collagen.马凡综合征中的主动脉瘤:胶原蛋白超微结构和组成的变化。
J Anat. 1979 Oct;129(Pt 3):645-57.