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Published on: September 15, 2017
Hypertension in children with congenital adrenal hyperplasia
Nadia Maccabee-Ryaboy1, William Thomas2, Jennifer Kyllo3
1University of Minnesota Masonic Children's Hospital, Minneapolis, MN, USA.
Insights
Children with congenital adrenal hyperplasia (CAH) have increased hypertension risk, particularly those on fludrocortisone and with oversuppressed 17-OHP levels. Incidence varies by CAH subtype and sex, with salt-wasting forms showing higher rates.
Area of Science:
- Pediatric Endocrinology
- Cardiovascular Health in Genetic Disorders
Background:
- Congenital adrenal hyperplasia (CAH) presents diverse clinical phenotypes, with varying estimates of hypertension incidence.
- Risk factors for hypertension in pediatric CAH patients remain incompletely understood.
Purpose of the Study:
- To estimate hypertension incidence stratified by CAH subtype and sex.
- To investigate associations between hypertension and body mass index, hydrocortisone, and fludrocortisone use.
Main Methods:
- Longitudinal chart review of 180 pediatric CAH patients (1970-2013).
- Hypertension defined as blood pressure ≥95th percentile on at least three occasions.
- Analysis included CAH subtype (salt-wasting vs. simple virilizing) and medication use.
Main Results:
- Hypertension incidence was higher in salt-wasting CAH (58%) than simple-virilizing CAH (35%).
- Children on fludrocortisone had significantly higher hypertension rates (55% vs. 31%).
- Oversuppression (17-OHP < 400 ng/dl) was associated with increased hypertension, especially in salt-wasting males on fludrocortisone.
Conclusions:
- Pediatric CAH patients face elevated hypertension risk compared to the general population.
- Fludrocortisone use and oversuppression are linked to higher hypertension incidence in CAH.
- Hypertension risk and timing differ significantly by CAH subtype and sex.
Objectives:
Estimates of high blood pressure (BP) incidence in children with congenital adrenal hyperplasia (CAH) vary widely; risk factors are poorly understood. We estimated incidence of hypertension by CAH subtype and sex, and assessed its association with body mass index, hydrocortisone and fludrocortisone.
Design:
Longitudinal.
Patients:
Chart review of 180 paediatric CAH patients (120 salt wasting; 60 simple virilizing; 93 females) seen from 1970 to 2013.
Measurements:
High BP was diagnosed by diastolic or systolic blood pressure measurement ≥95th percentile for age, sex and height; hypertension was diagnosed with high BP on at least three clinic visits.
Results:
Children with classic CAH who received fludrocortisone had a significantly higher rate of hypertension (55% vs 31%) than those who did not. Hypertension incidence was higher in salt-wasting CAH (58%) than in simple-virilizing CAH (35%). Hypertension first occurred before age 5 years in 91% of salt-wasting males and 50% of cases in salt-wasting females; most simple-virilizing cases occurred during ages 10-18 years. Rates of hypertension were higher in children who had three or more measurements with 17-OHP < 400 ng/dl (12·12 nmol/l), and this difference was significant in salt-wasting males. Children on fludrocortisone who had three or more readings of 17-OHP < 400 ng/dl (12·12 nmol/l) had a significantly higher rate of hypertension than those who did not. Hydrocortisone dose was not associated with hypertension.
Conclusion:
Children with CAH are at higher risk for hypertension than the general paediatric population, and incidence differs by sex and CAH subtype. Hypertension was higher in children on fludrocortisone and who were oversuppressed.
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