Context: Reports on mortality in @entity1 with @entity774 ( @entity774 ) are lacking. Objective: To study mortality and causes of @entity204 in @entity774 . Design, Setting and @entity1 : We studied @entity1 with @entity774 (21-hydroxylase deficiency, n=588; @entity4414 mutations known, >80%), and compared them with controls (n=58800). Data were derived through linkage of national population-based registers. Main Outcome Measures: Mortality and causes of @entity204 . Results: The mean age of @entity204 was 41.2  26.9 years in @entity774 @entity1 and 47.7  27.7 years in controls (P<0.001). Among @entity774 @entity1 23 (3.9%) had deceased compared to 942 (1.6%) of controls. The hazard ratio (and 95% confidence interval) of @entity204 was 2.3(1.2-4.3) in @entity774 males and 3.5(2.0-6.0) in @entity774 females. Including only @entity1 born 1952-2009, gave similar total results but only @entity1 with salt-wasting or with unclear phenotype had an increased mortality. The causes of @entity204 in @entity774 @entity1 were @entity774 (42%), cardiovascular (32%), @entity5 (16%), and suicide (10%). There were seven additional deaths in @entity774 individuals with incomplete or reused personal identification number that could not be analyzed using linkage of registers. Of the latter all except one were deceased before the introduction of neonatal screening in 1986 and most of them in the first weeks of life, probably in an @entity774 . Conclusions: @entity774 is a potentially lethal condition and was associated with excess mortality due to @entity774 . The salt-wasting phenotype seemed to have worse outcome also in @entity1 and adults due to @entity774 and not only before the introduction of neonatal screening.

Increased mortality in XXXX with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

@entity1 :: ('9606', 'Species') :: ['children', 'patients', 'Participants'] @entity4414 :: ('1589', 'Gene') :: ['CYP21A2'] @entity5 :: ('MESH:D009369', 'Disease') :: ['cancer'] @entity204 :: ('MESH:D003643', 'Disease') :: ['death'] @entity774 :: ('MESH:D000312', 'Disease') :: ['congenital adrenal hyperplasia', 'adrenal crisis', 'CAH']
@entity1 :: (9606,Species) :: ['patients']
