MINIPUBERTY OF INFANCY AND ADOLESCENT PUBERTAL FUNCTION IN ADRENAL HYPOPLASIA CONGENITA

An infant and his uncle, both with adrenal hypoplasia congenita, share d the same DAXI mutation. The adolescent uncle had hypogonadotropic hy pogonadism, but the infant had a normal minipuberty of infancy. These observations suggest differences in the physiologic mechanisms regulat ing the hypothalamic-pituitary-gonadal axis in infancy and adolescence .