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Genetic and Rare Diseases Information Center (GARD)

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Late-onset congenital adrenal hyperplasia


Other Names for this Disease

  • Attenuated congenital adrenal hyperplasia
  • LOCAH
  • NCCAH
  • Non classic congenital adrenal hyperplasia
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

I have been diagnosed with polycystic ovarian syndrome (PCOS), but the medication I am taking does not seem to be working. I recently learned that late-onset congenital adrenal hyperplasia can be misdiagnosed as PCOS in some women. Can you help me find information on PCOS and late-onset congenital adrenal hyperplasia?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

Can polycystic ovarian syndrome (PCOS) be distinguished from late-onset congenital adrenal hyperplasia (LOCAH)?

It can be difficult to determine whether a person has PCOS or LOCAH because the symptoms for these conditions are very similar. However, people with LOCAH usually have high levels of 17-hydroxyprogesterone in their blood.[1]
Last updated: 11/10/2008

How is late-onset congenital adrenal hyperplasia (LOCAH) diagnosed?

LOCAH can be diagnosed with an adrenocorticotropic hormone (ACTH) stimulation test. This test involves measuring the concentration of 17-hydroxyprogesterone (17-OHP) in the blood before ACTH is administered and 60 min after ACTH is given. People with LOCAH typically have 60-min stimulated 17-OHP values between 1,000 and 10,000 ng/dL.[2]
Last updated: 11/10/2008

How is late-onset congenital adrenal hyperplasia (LOCAH) treated?

The glucocorticoid called dexamethasone is often used to treat individiduals with LOCAH. Dexamethasone can treat irregular menstruation, acne, and excess body hair (hirsutism).[2]
Last updated: 11/10/2008

Where can I find additional information on polycystic ovarian syndrome (PCOS) and late-onset congenital adrenal hyperplasia (LOCAH)?

You can find links to more resources on the GARD Web pages for PCOS and LOCAH. In addition, we have located several full-text articles that discuss this topic. These articles are listed in PubMed, a searchable database of medical literature.

1. New MI. Extensive clinical experience: nonclassical 21-hydroxylase deficiency. J Clin Endocrinol Metab. 2006 Nov;91(11):4205-14. Epub 2006 Aug 15.

2. Azziz R, Sanchez LA, Knochenhauer ES, Moran C, Lazenby J, Stephens KC, et. al. Androgen excess in women: experience with over 1000 consecutive patients. J Clin Endocrinol Metab. 2004 Feb;89(2):453-62.

3. Moran C, Azziz R, Weintrob N, Witchel SF, Rohmer V, Dewailly D, et. al. Reproductive outcome of women with 21-hydroxylase-deficient nonclassic adrenal hyperplasia. J Clin Endocrinol Metab. 2006 Sep;91(9):3451-6. Epub 2006 Jul 5.
Last updated: 11/10/2008

References
  • Azziz R, Sanchez LA, Knochenhauer ES, Moran C, Lazenby J, Stephens KC, et. al. Androgen excess in women: Experience with over 1000 consecutive patients. J Clin Endocrinol Metab. 2004 Feb;89(2):453-62..
  • New MI. Extensive clinical experience: Nonclassical 21-hydroxylase deficiency. J Clin Endocrinol Metab. 2006 Nov;91(11):4205-14. Epub 2006 Aug 15. .
Other Names for this Disease
  • Attenuated congenital adrenal hyperplasia
  • LOCAH
  • NCCAH
  • Non classic congenital adrenal hyperplasia
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.