Search results for "Elastic fiber"

2018

The cutis laxa syndromes are multisystem disorders that share loose redundant inelastic and wrinkled skin as a common hallmark clinical feature. The underlying molecular defects are heterogeneous and 13 different genes have been involved until now, all of them being implicated in elastic fiber assembly. We provide here molecular and clinical characterization of three unrelated patients with a very rare phenotype associating cutis laxa, facial dysmorphism, severe growth retardation, hyperostotic skeletal dysplasia and intellectual disability. This disorder called Lenz-Majewski syndrome (LMS) is associated with gain of function mutations in PTDSS1, encoding an enzyme involved in phospholipid …

Elastic Fiber Density in Large and Small Airways and Parenchyma in Lungs of Non-Smokers, Smokers and COPD Patients.

2009

Identification of circumferential regional heterogeneity of ascending thoracic aneurysmal aorta by biaxial mechanical testing

2019

Abstract Ascending thoracic aortic aneurysm (ATAA) in patients with bicuspid aortic valve (BAV) can present an asymmetrical aortic dilatation compared with patients with tricuspid aortic valve (TAV). This pattern of aneurysm dilatation led us to hypothesize that biomechanical differences likely induced by regional heterogeneity of material properties can underlie the observed asymmetric enlargement discrepancies between BAV ATAA and TAV ATAA. This study aimed to characterize the mechanical properties and associated aortic tissue stiffness changes along the circumferential direction of aortic rings collected from surgically-repaired patients with ATAA. Biaxial material testing was performed …

Functional Morphology of Human Arteries During Fetal and Postnatal Development

1980

The equilibrium between the distending force of the blood pressure and the arterial wall is determined essentially by a simple law of mechanics, the law of Laplace. According to this law, the total force or tension (T) in the vessel’s wall represents the product of the radius of the vessel (r) and the blood pressure (p), T = r.p. The law of Laplace may be used to estimate and compare the tension produced by blood pressure in vessels of different size and thereby determine the increase in functional load on arteries. Since tension increases not only with blood pressure but also with the radius of the vessel, the highest tension is presumably produced in the wall of the ascending aorta where …