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Congenital adrenal hyperplasia increases hypertension risk
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The risk for hypertension is increased in children with congenital adrenal hyperplasia, and the risk is more pronounced with increasing disease severity, study data show.
Kyriakie Sarafoglou, MD, of the Leo Fung Center for Congenital Adrenal Hyperplasia and Disorders of Sex Development at the University of Minnesota Masonic Children’s Hospital in Minneapolis, and colleagues evaluated 180 pediatric patients with congenital adrenal hyperplasia (CAH) seen from 1970 to 2013 to determine the incidence of hypertension by CAH subtype and sex.
Researchers divided participants into two groups to examine changes in diagnosis and treatment over time: those born between 1970 and 1994 (pre-newborn screening for CAH) and between 1995 and 2013 (after newborn screening for CAH was initiated in Minnesota). Participants were also divided into groups based on CAH subtype: salt-wasting CAH (more severe; n = 120) and simple virilizing CAH (less severe; n = 60). Rates of hypertension were analyzed in participants with CAH prescribed fludrocortisone (n = 144) compared with those who were not (n = 36).
Among all participants, 69% were followed from CAH diagnosis. Among participants with salt-wasting CAH, 88% were diagnosed before age 1 year compared with 26% of participants with simple virilizing CAH.
Hydrocortisone dose decreased throughout the study period in all participants (P = .008), but those born after 1994 received lower doses overall (P < .001).
Among participants with at least three measurements of 17-hydroxyprogesterone (17-OHP), readings of 17-OHP less than 400 ng/dL, indicating oversuppression, were found in 80% of participants with salt-wasting CAH and 60% of participants with simple virilizing CAH.
The rate of hypertension was 60% in boys with salt-wasting CAH; 91% of cases occurred before age 5 years. A similar rate was found among girls, but only 50% of cases occurred before age 5 years. Girls with simple virilizing CAH had a higher crude rate of hypertension (48%) compared with boys (21%; P = .025). Blood pressure fluctuated between normal and hypertensive levels in 65% of participants, and 35% were normotensive.
CAH severity was positively related with rates of hypertension, with 58% of participants with salt-wasting CAH diagnosed with hypertension compared with 35% with simple virilizing CAH (P = .004). Participants with three or more readings of 17-OHP less than 400 ng/dL were more likely to experience hypertension compared with those with fewer than three readings at that level.
Fludrocortisone was prescribed in 80% of participants, including all participants with salt-wasting CAH. A higher rate of hypertension was found among participants prescribed fludrocortisone (55%) compared with those who were not (31%).
“The high rate of hypertension in children with classic CAH suggests that current management is suboptimal as shown by the association of hypertension with both 17-OHP oversuppression and fludrocortisone,” the researchers wrote. “Although levels of daily glucocorticoid dose have been reduced over the past 4 decades, our study found that hypertension rates have not decreased, suggesting other factors may play a role, such as glucocorticoid receptor sensitivity and noncircadian glucocorticoid administration.” – by Amber Cox
Disclosure: The researchers report no relevant financial disclosures.
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