21-hydroxylase deficiency surgery

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Congenital adrenal hyperplasia main page

Overview

Classification

21-hydroxylase deficiency
11β-hydroxylase deficiency
17 alpha-hydroxylase deficiency
3 beta-hydroxysteroid dehydrogenase deficiency
Cytochrome P450-oxidoreductase (POR) deficiency (ORD)
Lipoid congenital adrenal hyperplasia

Differential Diagnosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohammed Abdelwahed M.D[2]

Overview

Reconstructive surgery and Surgical adrenalectomy.

Surgery

Reconstructive surgery

reconstructive gynecologic surgery as adolescents or adults, including clitoroplasty and vaginoplasty.[1]

Surgical adrenalectomy

The major benefit is lowering adrenal androgen and progesterone secretion. However, It increases dependency on glucocorticoid and mineralocorticoid replacement therapy.[2]

References

  1. Premawardhana LD, Hughes IA, Read GF, Scanlon MF (1997). "Longer term outcome in females with congenital adrenal hyperplasia (CAH): the Cardiff experience". Clin Endocrinol (Oxf). 46 (3): 327–32. PMID 9156043.
  2. Crocker MK, Barak S, Millo CM, Beall SA, Niyyati M, Chang R; et al. (2012). "Use of PET/CT with cosyntropin stimulation to identify and localize adrenal rest tissue following adrenalectomy in a woman with congenital adrenal hyperplasia". J Clin Endocrinol Metab. 97 (11): E2084–9. doi:10.1210/jc.2012-2298. PMC 3485588. PMID 22904181.