Endocrine Abstracts

JOINT3960

We report a case of a 4-year-old patient with salt wasting congenital adrenal hyperplasia who developed prepubertal gynecomastia as a symptom of insufficient substitutive therapy. The patient was treated since the 6^th day of life with hydrocortisone (HC) and fludrocortisone (FC). At the age of 4years the dose of HC was 10mg/square m. Laboratory tests confirmed elevated levels of adrenocorticotropic hormone (ACTH), androstenedione and 17-hydroxyprogesterone (17-OHP), and 24-hours urine collection also revealed not enough suppression of androgens and 17-OHP metabolites. Following an increase in hydrocortisone dosage, the gynecomastia completely regressed during 3 months. In the described case, TART and other estrogen-secreting tumors were considered in the differential diagnosis, but the complete resolution of gynecomastia after increasing the dose of hydrocortisone indicates that the cause was insufficient replacement. Gynecomastia as a dominant symptom of insufficient replacement therapy in congenital adrenal hyperplasia in a boy is rarely described, and premature pubarche is much more frequently observed. A probable explanation may be the increased aromatization of adrenal androgens to estrogens in prepubertal patients.