PURPOSE: @entity20117 @entity21559 ( @entity20117 @entity21559 ) @entity2783 is a phenotypically diverse disease with symptoms ranging from predominant @entity16 to exclusive @entity299 . The aims of this study were to determine the dispersion of the two types of fibrils found in Swedish @entity20117 @entity21559 @entity1 -Type A consisting of a mixture of truncated and full length @entity20117 fibrils and type B fibrils consisting of full length fibrils, and to estimate the severity of @entity299 in relation to fibril composition and sex. MATERIAL AND METHODS: Echocardiographic data were analysed in 107 Swedish @entity20117 @entity21559 @entity1 with their fibril composition determined as either @entity1129 or type B. Measurements of left ventricular (LV) dimensions and evaluation of systolic and diastolic function including speckle tracking derived strain were performed. @entity1 were grouped according to fibril type and sex. Multivariate linear regression was utilised to determine factors of significant impact on LV thickness. RESULTS: There was no significant difference in proportions of the two types of fibrils between @entity1 and @entity1 . In @entity1 with @entity1129 fibrils, @entity1 had significantly lower median septal (p = 0.007) and posterior wall thicknesses (p = 0.010), lower median LV mass indexed to height (p = 0.008), and higher septal strain (p = 0.037), as compared to males. These differences were not apparent in @entity1 with type B fibrils. Multiple linear regression analysis revealed that fibril type, sex and age all had significant impact on LV septal thickness. CONCLUSION: This study demonstrates a clear difference between sexes in the severity of @entity299 in @entity20117 @entity21559 @entity2783 @entity1 . Even though @entity1129 fibrils were associated with more advanced @entity299 compared to type B, @entity1 with @entity1129 fibrils generally developed less cardiac infiltration than @entity1 . The differences may explain the better outcome for liver transplanted late-onset female @entity1 compared to males.

Amyloid Cardiomyopathy in Hereditary @entity20117 XXXX Amyloidosis - Impact of Sex and Amyloid Fibril Composition.

@entity1 :: ('9606', 'Species') :: ['women', 'Patients', 'patients', 'men'] @entity21559 :: ('p|SUB|V|30|M', 'ProteinMutation') :: ['V30M'] @entity2783 :: ('MESH:D000686', 'Disease') :: ['amyloidosis'] @entity16 :: ('MESH:D009422', 'Disease') :: ['neuropathy'] @entity1129 :: ('MESH:D001260', 'Disease') :: ['type A'] @entity299 :: ('MESH:D006331', 'Disease') :: ['cardiac manifestations', 'amyloid heart disease', 'cardiac dysfunction'] @entity20117 :: ('7276', 'Gene') :: ['ATTR', 'Transthyretin']
@entity21559 :: (p|SUB|V|30|M,ProteinMutation) :: ['V30M']
