OBJECTIVE: To determine mortality in patients with congenital adrenal hyperplasia (CAH) compared with that in the general population. DESIGN: 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.
OBJECTIVE: To determine mortality in patients with congenital adrenal hyperplasia (CAH) compared with that in the general population. DESIGN: 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: E Charmandari; E J Lichtarowicz-Krynska; P C Hindmarsh; A Johnston; A Aynsley-Green; C G Brook Journal: Arch Dis Child Date: 2001-07 Impact factor: 3.791
Authors: Jonathan Weber; Veeraya K Tanawattanacharoen; Amy Seagroves; Mark C Liang; Christina M Koppin; Heather M Ross; Tania A S S Bachega; Mitchell E Geffner; Monica Serrano-Gonzalez; Gagandeep Bhullar; Mimi S Kim Journal: J Clin Endocrinol Metab Date: 2022-01-01 Impact factor: 6.134
Authors: Alaina P Vidmar; Jonathan F Weber; Roshanak Monzavi; Christina M Koppin; Mimi S Kim Journal: J Pediatr Endocrinol Metab Date: 2018-01-26 Impact factor: 1.520
Authors: R Louise Rushworth; Henrik Falhammar; Craig F Munns; Ann M Maguire; David J Torpy Journal: Int J Endocrinol Date: 2016-01-06 Impact factor: 3.257