What is the role of puberty in the management of congenital adrenal hyperplasia (CAH)?

Updated: Oct 06, 2020
  • Author: Thomas A Wilson, MD; Chief Editor: Sasigarn A Bowden, MD  more...
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Answer

Some patients develop precocious puberty, perhaps secondary to the advanced growth and skeletal maturation that occurs with androgen exposure. This may be treated with GnRH analogue therapy.

As puberty progresses, monitor for adrenal rests within the gonads. If ACTH is inadequately suppressed, these maybe mistaken for gonadal tumors and may cause gonadal pain. Adrenal rests are more commonly found in the testes than in the ovaries.

As adulthood approaches, vaginal adequacy should be assessed because many females with adrenal hyperplasia suffer from dyspareunia due to vaginal stenosis.

The Endocrine Society's 2010 clinical practice guidelines recommend that when patients with CAH reach adulthood, they should be assessed for known complications of CAH. [7]


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