title: Mortality in patients with congenital adrenal hyperplasia: a cohort study.
abstract: OBJECTIVE
  To determine mortality in patients with congenital adrenal hyperplasia (CAH) compared with that in the general population.
  
  
  METHODS
  We identified 333 children with CAH, treated at several pediatric endocrinology departments in the United Kingdom since 1964, and monitored their mortality to mid 1996. Standardized mortality ratios were calculated, comparing mortality in the cohort with that in the general population, adjusted for sex, age, and calendar period.
  
  
  RESULTS
  All-cause mortality in the cohort was 3 times that expected. Mortality was significantly increased at ages 1 to 4 years (standardized mortality ratio = 18.3) but not at older ages and was significantly increased in patients of Indian-subcontinent ethnicity (standardized mortality ratio = 20.4), particularly in girls. From case notes and death certificates, it appears that most deaths were caused by adrenal crisis, often after infection.
  
  
  CONCLUSIONS
  Although survival of patients with CAH has greatly improved since steroid therapy has been used, this disease can still have fatal consequences. The high mortality rate in Indian ethnic girls may well reflect lack of parental acceptance and understanding of the disease, as well as of the action required when their child becomes acutely ill. Better communication with and education of parents of children with CAH, especially those from immigrant ethnic minorities, is important.
authors.affiliation: Epidemiological Monitoring Unit, London School of Hygiene and Tropical Medicine, England.
authors.forename: A J
authors.initials: AJ
authors.lastname: Swerdlow
authors.forename: C D
authors.initials: CD
authors.lastname: Higgins
authors.forename: C G
authors.initials: CG
authors.lastname: Brook
authors.forename: D B
authors.initials: DB
authors.lastname: Dunger
authors.forename: P C
authors.initials: PC
authors.lastname: Hindmarsh
authors.forename: D A
authors.initials: DA
authors.lastname: Price
authors.forename: M O
authors.initials: MO
authors.lastname: Savage
mesh_terms: D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000401:mortality*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D006899:Mixed Function Oxygenases / Q000172:deficiency; D015996:Survival Rate; D006113:United Kingdom / Q000453:epidemiology
chemical_list: D006899:Mixed Function Oxygenases