XXXX : structural, biochemical, and mechanical studies of the aortic media.

@entity1 @entity455 @entity2362 @entity13403 @entity1614 @entity439 @entity74 @entity2333 @entity65
An intrinsic defect in the aortic media in six @entity1 with @entity1614 , who died of @entity65 at an average age of 32 years, has been delineated by correlated morphologic, biochemical, and mechanical studies. The findings in the Marfan aortas have been compared with those in age- and sex-matched controls, who died of unrelated diseases without significant @entity2333 , and in three @entity1 with dissecting @entity439 of non-Marfan origin. The results showed that there was a significant reduction in the tensile strength of the aorta in @entity1614 . This finding was correlated by scanning electron microscopy with structural alterations of the medial elastic fibers, including enlarged interlaminar spaces and loss of interlaminar elastic fibrils. No structural alterations were identified in collagen fibers. Biochemical analyses of the aortic media revealed a substantial reduction in aortic @entity2362 content. Furthermore, the @entity13403 content of the isolated @entity2362 was reduced by approximately 50%. No changes were detected in the composition or solubility of the medial collagen. In contrast to Marfan aortas, the @entity2362 and collagen contents of the dissecting @entity439 of non-Marfan origin were similar to those of the controls. These findings suggest that the @entity455 in @entity1614 may be based on a @entity74 affecting @entity2362 fibrillogenesis.
