Progress of minerocorticoid therapy in 21-hydroxylase deficiency
10.3760/cma.j.cn101070-20240629-00405
- VernacularTitle:21-羟化酶缺乏症盐皮质激素治疗方案进展
- Author:
Luyue SUN
1
;
Hui YAO
1
;
Tian LAN
1
Author Information
1. 华中科技大学同济医学院附属武汉儿童医院遗传代谢内分泌科，武汉 430016
- Publication Type:Journal Article
- Keywords:
21-hydroxylase deficiency;
Mineralocorticoid;
Plasma renin activity level;
Blood pressure;
Electrolyte
- From:
Chinese Journal of Applied Clinical Pediatrics
2025;40(5):393-396
- CountryChina
- Language:Chinese
-
Abstract:
21-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.
