This protocol covers classic congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency in the pediatric population — growing children under 18 years of age, including the newborn and early infancy period. It represents the first-line evidence-based approach for this age group.
Management is built around maintenance corticosteroid replacement therapy; the newborn and early infancy period requires additional supplementation beyond the core regimen. Certain glucocorticoid formulations are explicitly to be avoided in growing patients.
Full regimen details — including agents, dosing, and sequencing — are available in the structured protocol below.
The target is upper normal to mildly elevated serum 17-hydroxyprogesterone and androstenedione levels, alongside nearly normal growth and development. Complete suppression of 17-hydroxyprogesterone is not a goal — it indicates overtreatment.
DOI: 10.1210/jc.2018-01865
In growing individuals with classic CAH, we recommend maintenance therapy with hydrocortisone (HC).
In growing individuals with CAH, we recommend against the use of oral HC suspension and against the chronic use of long-acting potent GCs.
In the newborn and in early infancy, we recommend using fludrocortisone and sodium chloride supplements to the treatment regimen.
Acceptably treated patients with CAH generally have upper normal to mildly elevated 17OHP and androstenedione levels when measured in a consistent manner.
Complete suppression of serum 17OHP level is not a treatment goal but instead indicates overtreatment.
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