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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.

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

Terminology
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● 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"

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

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

Clinical Features
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● 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
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Pediatrics 2006;118:e488

End of Ovary - nontumor > Gonadal dysgenesis > Female pseudohermaphroditism


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