OBJECTIVES: Assess the impact of @entity454 ( @entity740 stage 5 (CKD5)) on cardiovascular outcomes in @entity1 with @entity2314 on enzyme replacement therapy. BACKGROUND: @entity2314 , an @entity2315 , causes @entity1121 and @entity65 . METHODS: Cardiac and renal function of 25 male @entity1 with @entity2314 were analysed at 0, 1, 2, 5, 7 and 10   years after initiation of treatment. @entity1 were grouped at baseline into those with CKD5 (n=10) and those without (n=15). ECG and echocardiography were performed 6 and 12 monthly, respectively, while renal function was measured yearly. RESULTS: After 10   years of treatment, cardiac and renal function in non-CKD5 @entity1 remained unchanged. In contrast, CKD5 was associated with worse baseline cardiac parameters and progressive @entity1184 . LV mass index grew by 35.4  31.8   g/m(2.7) in CKD5 versus 5.7  7.9   g/m(2.7), p=0.044 in non-CKD5, predominantly due to increased interventricular septal wall thickness (7.7  5.5   mm vs 1.3  1.7   mm, p=0.003). @entity65 , including @entity944 , @entity943 and pacing device insertion, occurred in 100% @entity1 with CKD5 (21 events) and 26% non-CKD5 @entity1 (7 events), p<0.0001. Additionally, estimated LV filling pressure (E/Ea) was significantly higher in @entity1 having cardiovascular events (21.1  7.7 vs 12.5  4.5, p=0.008) irrespective of renal function. CONCLUSIONS: @entity454 was the strongest indicator of @entity65 progression in @entity2314 . Enzyme replacement initiated prior to CKD5 was associated with stability in cardiac and @entity8 while @entity1 with @entity703 . Additionally, E/Ea >= 15 may predict risk of @entity299 .

Cardiovascular outcomes in XXXX are linked to severity of @entity740 .

@entity1 :: ('9606', 'Species') :: ['Patients', 'patients'] @entity2315 :: ('MESH:D016464', 'Disease') :: ['X-linked lysosomal storage disease'] @entity8 :: ('MESH:D007674', 'Disease') :: ['renal disease'] @entity943 :: ('MESH:D001145', 'Disease') :: ['arrhythmia'] @entity944 :: ('MESH:D003645', 'Disease') :: ['sudden death'] @entity1184 :: ('MESH:D006984', 'Disease') :: ['LV hypertrophy'] @entity703 :: ('MESH:D007680', 'Disease') :: ['CKD5 showed ongoing deterioration'] @entity740 :: ('MESH:D051436', 'Disease') :: ['chronic kidney disease'] @entity2314 :: ('MESH:D000795', 'Disease') :: ['Fabry disease'] @entity299 :: ('MESH:D006331', 'Disease') :: ['cardiac events'] @entity1121 :: ('MESH:D002312', 'Disease') :: ['hypertrophic cardiomyopathy'] @entity65 :: ('MESH:D002318', 'Disease') :: ['cardiovascular dysfunction', 'Cardiovascular events', 'cardiovascular disease'] @entity454 :: ('MESH:D007676', 'Disease') :: ['end-stage renal disease', 'End-stage renal disease']
@entity2314 :: (MESH:D000795,Disease) :: ['Fabry disease']
