Sólyom J
II. Gyermekklinika, Semmelweis Orvostudományi Egyetem, Budapest.
Orv Hetil. 1992 Jun 21;133(25):1539-44; 1547.
This review deals with the current problems in the management of classical 21-hydroxylase deficiency from the fetal life to the puberty. The clinical consequences of 21-hydroxylase deficiency reflect the disordered physiology--impaired secretion of glucocorticoids and mineralocorticoids, and excessive secretion of androgens. Current therapy is intended to correct the disordered physiology by replacing mineralocorticoid and glucocorticoid hormones, thereby reducing the ACTH-driven increase in adrenal androgen secretion. Treated patients should expect a normal life span and reproductive potential. This can be achieved by careful attention to regular measurements of clinical parameters and biochemical indices of control.
本综述探讨了从胎儿期到青春期经典型21-羟化酶缺乏症管理中的当前问题。21-羟化酶缺乏症的临床后果反映了生理紊乱——糖皮质激素和盐皮质激素分泌受损，以及雄激素分泌过多。目前的治疗旨在通过补充盐皮质激素和糖皮质激素来纠正生理紊乱，从而减少促肾上腺皮质激素驱动的肾上腺雄激素分泌增加。接受治疗的患者有望拥有正常的寿命和生殖潜力。这可以通过密切关注定期测量临床参数和控制的生化指标来实现。
