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Correspondence to Author: Mikole Keisch, 

Medizinische Klinik IV, Klinikum der Universität München, 80336 Munich, Germany.

INTRODUCTION: 
Individuals who have 21-hydroxylase deficiency-related classic congenital adrenal hyperplasia (CAH) need cortisol replacement therapy for the rest of their lives. Patients with the most severe form die in the first few weeks of life from salt-wasting crisis if treatment is not received. Nearly all patients survive because newborn screening programs are now accessible in almost all Western countries and adrenal replacement medication was first introduced 70 years ago. Even after seven decades, the majority of patients still receive hydrocortisone three times a day as part of their medication. The dual therapeutic challenges in CAH are hormone replacement and excess adrenal androgen regulation, especially at night to avoid the early morning surge in adrenal androgens caused by adrenocorticotropin (ACTH). Supraphysiological levels of glucocorticoids are required to achieve the latter, and in certain situations, these doses must be taken in a reverse circadian therapy regimen, with the highest dose given right before bed. Longer-acting synthetic glucocorticoids are frequently used in adult treatment to promote compliance and manage hyperandrogenemia. These medications are given once or twice daily.

Citation:

Mikole Keisch. A Novel Therapeutic Approach for Congenital Adrenal Hyperplasia: Block and Replace?. Journal of Clinical Endocrinology and Metabolism 2024.