BACKGROUND: @entity3606 is a rare but serious @entity69817 leading to substantial morbidity and early mortality due to @entity943 and @entity296 . The X-linked nature of inheritance accounts for reported differences in phenotypic severity between @entity1 and @entity1 . The rarity of @entity3606 has limited understanding of the complete phenotype. Clinical estimates of ages of disease onset and survival based on gender have not been published. METHODS AND RESULTS: We present data on 82 @entity1 with @entity3606 from 36 families, the largest series to date. @entity1 were severely affected with @entity73 (100%), @entity1121 (88%), and @entity425 (80%). @entity1 had a high morbidity and were unlikely to reach the age of 25 years without a cardiac transplantation. @entity1 were less severely affected but reported higher than expected levels of cognitive (47%) and skeletal muscle complaints (50%) and manifesting an equal prevalence of @entity384 and @entity1121 . Combining our data with that of 63 other @entity3606 case reports in the literature, the average ages of first symptom, cardiac transplantation, and @entity204 were 12.1, 17.9, and 19.0 years in @entity1 and 27.9, 33.7, and 34.6 years in @entity1 , respectively. CONCLUSION: These data more broadly illuminate the @entity3606 phenotype and should prove useful to physicians working with and providing genetic counseling to families with @entity3606 . @entity1 with @entity3606 present with clinical symptoms and events approximately 15 years after @entity1 and report a higher proportion of cognitive and skeletal muscle problems than previously recognized.

Natural history of XXXX .

@entity1 :: ('9606', 'Species') :: ['women', 'patients', 'Women', 'Men', 'men'] @entity296 :: ('MESH:D009202', 'Disease') :: ['cardiomyopathy'] @entity73 :: ('MESH:D003072', 'Disease') :: ['cognitive disabilities'] @entity384 :: ('MESH:D002311', 'Disease') :: ['dilated cardiomyopathy'] @entity425 :: ('MESH:D018908', 'Disease') :: ['muscle weakness'] @entity3606 :: ('MESH:D052120', 'Disease') :: ['Danon disease'] @entity1121 :: ('MESH:D002312', 'Disease') :: ['hypertrophic cardiomyopathy'] @entity943 :: ('MESH:D001145', 'Disease') :: ['arrhythmia'] @entity69817 :: ('MESH:C538496', 'Disease') :: ['cardiac and skeletal myopathy'] @entity204 :: ('MESH:D003643', 'Disease') :: ['death']
@entity3606 :: (MESH:D052120,Disease) :: ['Danon disease']
