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Definition / generalCite this page: Al-Hussain T. Congenital adrenal hyperplasia. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/testisCAH.html. Accessed January 20th, 2021.
Definition / general
- Autosomal recessive, due to various enzymatic defects that cause different patterns of synthesis of glucocortical, mineralocorticoid and sex hormone synthesis
- Genetic males have cryptorchidism, viable Wolffian duct structures, female or ambiguous genitalia, no Müllerian duct structures
- Testes resemble cryptorchid testes
- May have bilateral Leydig cell hyperplasia with deficiencies of 21-hydroxylase, 11β hydroxylase, 17α hydroxylase, 3β hydroxysteroid dehydrogenase
- Treat with corticosteroids or surgical excision of testes