线粒体脑病、乳酸中毒和卒中样发作患者的主动脉破裂
Aortic rupture in mitochondrial encephalopathy,lactic acidosis,and stroke-like episodes作者机构：4-420 College of Physicians and Surgeons 630 W 168th St New York NY 10032 United States Dr.
出 版 物：《世界核心医学期刊文摘（神经病学分册）》 (Digest of the World Core Medical Journals:Clinical Neurology)
年 卷 期：2006年第2卷第6期
页 面：19-19页
学科分类：1002[医学-临床医学] 100210[医学-外科学(含：普外、骨外、泌尿外、胸心外、神外、整形、烧伤、野战外)] 10[医学]
主 题：外科手术患者 主动脉破裂 线粒体脑病 卒中样发作 乳酸中毒 限制片段长度多态性分析 微血管病变 免疫组织化学染色 细胞色素C氧化酶 MELAS
摘 要：Background: Microangiopathy has been well described in the brain and muscle of patients with mitochondrial encephalopathy, lactic acidosis, and strokelike episodes (MELAS). Objective: To describe a patient with the common A3243G/MELAS point mutation who had aortic rupture and whose mother also died of large vessel rupture. Design: Case report. Setting: Collaboration between a primary care hospital and 2 academic tertiary care hospitals. Results: Histologically, there was marked disarray of the smooth muscle architecture of the aorta, and immunohistochemical staining with antibodies against the mitochondrial DNA-encoded cytochrome-C oxidase I subunit showed uniformly decreased immunostaining of the endothelial and smooth muscle cells of the aorta and vasa vasorum. Polymerase chain reaction and restriction fragment length polymorphism analysis showed that the mutation load was 40.5%in blood but 85.3%in the blood vessels. Conclusions: The severe vasculopathy in this patient is probably directly related to the high mutation load in the blood vessels. Although aortic rupture is an unusual manifestation of MELAS, it is an important potential complication in patients undergoing minor surgical procedures.
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