JCEM Case Reports Journal Article

Abiraterone in Classic Congenital Adrenal Hyperplasia

July 23, 2024

Results of Medical Therapy Before Adrenalectomy

 

Bronwyn G A Stuckey, Deila Dedic, Rui Zhang, Amira Rabbah, Adina F Turcu, Richard J Auchus
JCEM Case Reports, Volume 2, Issue 6, June 2024, luae077
https://doi.org/10.1210/jcemcr/luae077

Abstract

We present the case of a 20-year-old woman with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency, with uncontrolled hyperandrogenemia despite supraphysiological glucocorticoid therapy. We used abiraterone acetate, an inhibitor of the 17-hydroxylase/17,20-lyase enzyme, to suppress adrenal androgen synthesis and allow physiological glucocorticoid and mineralocorticoid therapy, as a proof-of-concept, before proceeding to bilateral adrenalectomy. We report the patient's clinical course, the changes in adrenal steroids, and the immunohistochemistry of the adrenals.

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