Histopathology of an Iliac Aneurysm in a Case of Menkes Disease

被引：8

作者：

Borges, Luciano De Figueiredo ^{[1
,2
]}

Martelli, Helene ^{[3
]}

Fabre, Monique ^{[4
]}

Touat, Ziad ^{[1
]}

Jondeau, Guillaume ^{[1
]}

Michel, Jean-Baptiste ^{[1
]}

机构：

[1] CHU Xavier Bichat, INSERM, U698, F-75018 Paris, France

[2] Univ Fed Sao Paulo, Ctr Electron Microscopy, Sao Paulo, Brazil

[3] Kremlin Bicetre Hosp, Dept Pediat Surg, Le Kremlin Bicetre, France

[4] Kremlin Bicetre Hosp, Dept Pathol, Le Kremlin Bicetre, France

来源：

PEDIATRIC AND DEVELOPMENTAL PATHOLOGY | 2010年 / 13卷 / 03期

关键词：

apoptosis; intraluminal thrombus; mucoid degeneration; smooth muscle cells; ABDOMINAL AORTIC-ANEURYSMS; NITRIC-OXIDE-SYNTHASE; MEDIAL DEGENERATION; MURAL THROMBUS; ARTERY ANEURYSMS; MUTATIONS; CELL; DISSECTIONS; INHIBITION; ACTIVATION;

D O I：

10.2350/08-08-0516.1

中图分类号：

R36 [病理学];

学科分类号：

100104 ;

摘要：

In Menkes disease, arterial tortuosity is frequent, whereas true aneurysms are rare. Here, we report aneurysmal pathology occurring in an infant with Menkes disease. An iliac aneurysm was diagnosed in a 2-month-old boy and attributed to Menkes syndrome on the basis of plasma copper deficiency. Samples of the aneurysmal wall were taken during surgery for histopathological analysis. As in other forms of aneurysm, the arterial wall was characterized by smooth muscle cell (SMC) disappearance, linked to SMC apoptosis and oxidative stress, areas of mucoid degeneration, and extracellular matrix breakdown, including disappearance of elastic fibers and presence of abnormal collagen.

引用

页码：247 / 251

页数：5

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