Home   Chapter Home   Jobs   Conferences   Fellowships   Books


Ovary - nontumor

Gonadal dysgenesis

Female pseudohermaphroditism

Reviewer: Mohiedean Ghofrani, M.D., (see Reviewers page)
Revised: 28 December 2011, last major update December 2011
Copyright: (c) 2002-2012, PathologyOutlines.com, Inc.


● A disorder of sex development (DSD) with female genotype (46,XX), female internal phenotype (two ovaries) but variable degrees of virilization


● In 2006 the International Consensus Conference on Intersex recommended using the designation "46,XX DSD" instead of the potentially pejorative and confusing term "female pseudohermaphroditism"


● Serum steroid measurements can confirm or rule out congenital adrenal hyperplasia in most 46,XX DSD cases


● Most virilized 46,XX infants have congenital adrenal hyperplasia (CAH)
● Non-CAH etiologies include gestational hyperandrogenism

Clinical Features

● In CAH, depending on the site of the steroid biosynthesis defect, there may be under- or over-production of mineralcorticoid, resulting in imbalances in serum electrolytes and blood pressure

Additional references

Pediatrics 2006;118:e488

End of Ovary - nontumor > Gonadal dysgenesis > Female pseudohermaphroditism

This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at [email protected] with any questions (click here for other contact information).