摘要21-羟化酶缺乏症是类固醇合成途径中酶的缺乏导致肾上腺皮质激素合成不足和雄激素过高引起的一种罕见病。主要以糖皮质激素和盐皮质激素替代治疗，目前糖皮质激素替代治疗的具体剂量和用法有指南做依据，但盐皮质激素剂量调整尚无详细依据。盐皮质激素替代不足会引起糖皮质激素剂量增加，造成医源性库欣综合征；用量过多又会引起水钠潴留，造成药物性高血压。故氟氢可的松的剂量调整和治疗监测仍需进一步探讨。现就21-羟化酶缺乏症盐皮质激素治疗方案及监测的进展进行综述。

abstracts21-hydroxylase deficiency (21-OHD) is a rare disease caused by insufficient adrenocortical hormone synthesis and hyperandrogen due to enzyme deficiency during steroid synthesis.Glucocorticoid and mineralocorticoid replacement therapies are main treatment method for 21-OHD.Currently, there are guidelines for the specific dosage and usage of the glucocorticoid replacement therapy, but no detailed evidence is offered for mineralocorticoid dose adjustment.Insufficient mineralocorticoid replacement can lead to an increase in the dose of glucocorticoids and further iatrogenic Cushing′s syndrome.Excessive dosage can result in sodium and water retention and further drug-induced hypertension.Therefore, the dose adjustment and treatment monitoring of Fludrocortisone still need to be further explored.In this article, the progress in mineralocorticoid treatment and monitoring for 21-OHD will be reviewed.