Wieczorek P, Riegel M B, Quattro L, DeMaio K
Johns Hopkins Hospital, Baltimore, USA.
AORN J. 1996 Dec;64(6):895-913; quiz 916-8, 921-2. doi: 10.1016/s0001-2092(06)63601-6.
Marfan's syndrome is an inherited, degenerative connective tissue disorder that affects many body systems (eg, skeletal, ocular, cardiovascular, cutaneous, pulmonary, abdominal, neurologic). The cause of Marfan's syndrome is unknown, but recent genetic studies have linked this disorder to chromosome 15q15-q21.3. The characteristics associated with Marfan's syndrome require a multidisciplinary approach to patient care. This article discusses one serious complication of Marfan's syndrome-aortic root dilatation- and composite graft repairs of ascending aortic aneurysms. Physicians and nurses must be more aware of Marfan's syndrome so that life-threatening medical conditions can be evaluated and followed by health care providers.
马方综合征是一种遗传性、退行性结缔组织疾病,会影响多个身体系统(如骨骼、眼部、心血管、皮肤、肺部、腹部、神经)。马方综合征的病因尚不清楚,但最近的基因研究已将这种疾病与15号染色体的15q15-q21.3区域联系起来。马方综合征相关的特征需要多学科方法来进行患者护理。本文讨论马方综合征的一种严重并发症——主动脉根部扩张,以及升主动脉瘤的复合移植物修复。医生和护士必须更加了解马方综合征,以便医疗保健提供者能够评估并跟踪可能危及生命的医疗状况。