Tamura K, Fukuda Y, Ferrans V J
Department of Pathology, Nippon Medical School, Tokyo, Japan.
J Heart Valve Dis. 1998 Jul;7(4):460-6.
The alterations observed in the elastic fibers of the perforated floppy mitral valve of a 68-year-old man are described. The patient had not history of infective endocarditis and no stigmata of Marfan syndrome or Ehlers-Danlos syndrome. Ultrastructurally, the elastic fibers in the area of the perforation showed unique, round-shaped expansion of the amorphous components, which lacked associated microfibrils. These features resembled those of the elastic fibers in animals treated with beta-aminopropionitrile, an inhibitor of lysyl oxidase. Aggregations of microfibrils without amorphous components were also observed. These abnormalities are considered to be related to the pathogenesis of the perforation in this valve.
描述了一名68岁男性穿孔性松弛二尖瓣弹性纤维的观察变化。该患者无感染性心内膜炎病史，无马凡综合征或埃勒斯-当洛综合征的体征。超微结构上，穿孔区域的弹性纤维显示无定形成分独特的圆形扩张，且缺乏相关微原纤维。这些特征类似于用β-氨基丙腈（一种赖氨酰氧化酶抑制剂）处理过的动物的弹性纤维。还观察到无微原纤维无定形成分的聚集。这些异常被认为与该瓣膜穿孔的发病机制有关。
