SUN-466 - Characteristics of Patients with Congenital Adrenal Hyperplasia in A Children'S Hospital

Since 2009, 79 patients with congenital adrenal hyperplasia (CAH) have been evaluated in a free-standing children’s hospital. Of those patients, 38 (48%) had non-classic CAH and 41 (52%) had classic CAH. Among patients with classic CAH, 34 (83%) had the salt-wasting (SW-CAH) type and 7 had the simple virilizing (SV-CAH).

Pharmacological treatment:
Among patients with NC-CAH, 55% were not treated with steroids. Of the 17 patients receiving steroids (45%), 15 were treated with hydrocortisone (HC), 1 with dexamethasone, and 1 with prednisone. Among the 41 patients with classic CAH, 35 (85%) were treated with hydrocortisone, 4 (1%) with dexamethasone, and 1 (0.25%) with prednisone. 1 patient with SV subtype was not treated with steroid. All patients with SW-CAH received fludrocortisone, as did the 3 out of 7 with SV-CAH.

Patients with NC-CAH received a mean hydrocortisone-equivalent (HC-E) dose of 10.1 mg/m2/day (with 1 mg of dexamethasone equivalent to 60 mg of HC and 1 mg of prednisone equivalent to 4 mg of HC). Patients with SW-CAH received a mean of 13.4 mg/m2/day of HC-E, and patients with SV-CAH a mean dose of 17.7 mg/m2/day.

Steroid levels:
The mean 17-hydroxyprogesterone (17OHP) was 3,100 ng/dL among patients with SW-CAH, and 2,102 ng/dL among patients with SV-CAH. Mean level of androstenedione (D4) was 158 ng/dL among patients with SW-CAH, and 206 ng/dL among patients with SV-CAH.

Conclusions:
Most of our patients with NC-CAH were not treated with steroids, as their bone age was not sufficiently advanced to compromise their predicted adult height, and female patients with signs of hyperandrogenism were adequately controlled with oral contraceptives.

Most patients with classic CAH had mean 17OHP and D4 levels above target goal ( < 1,000 ng/dL for 17OHP and < 50 ng/dL in prepubertal and < 200 ng/dL in pubertal patients for D4), despite being treated with supraphysiologic doses, defined as HC-E dose >11 mg/m2/day.

*Unless otherwise noted, all abstracts presented at ENDO must not be released to the press or the public until the date and time of presentation. For oral presentations, the abstracts are embargoed until the session begins. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO.*