280 patients (with 984 clinical visits and plasma renin measurements) with primary adrenal insufficiency recruited from local databases and the international congenital adrenal hyperplasia (CAH) registry (www.i-cah.org).
Congenital adrenal hyperplasia (CAH) due to steroid 11β-hydroxylase deficiency (11β-OHD) is a rare form of CAH associated with low renin hypertension, hypokalemia, hyperandrogenemia and ambiguous genitalia in affected females.
17-OHP is a diagnostic marker for CAH but opinions differ on the role of 17OHP or androstenedione in monitoring treatment with renin in the salt losing form.