Drug strategy for adolescent 21 hydroxylase deficiency
Management of adolescents with 21-hydroxylase deficiency(21-OHD)presents unique challenges because of poor adherence to medical therapy and the increase in drug metabolismand clearance rate.Rational use of adrenocortical hormone for adolescents with 21-OHD is emphasized to avoid excessive androgen and premature closure of epiphysis caused by insufficient glucocorticoids,and to avoid drug overdose resulting in secondary Cushing syndrome.It is very important to monitor the growth and development of adolescents and biomarkers,adjust the medication in time and find the complicating diseases correctly,such as testicular adrenal rest tumours(TARTs).There is no guideline or consensus on the application of growth hormones and aromatase inhibitors,and the indications for withdrawal of gonadotropin releasing hormone analogues in adolescent children,and individualized diagnosis and treatment is required.
21-hydroxylase deficiencyrecombinant human growth hormonearomatase inhibitorsgonadotropin releasing hormone analogues