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ACTH-independant macronodular adrenal hyperplasia: imaging findings of a rare condition : A case report.


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http://www.springerlink.com/content/b5508m0018201655/

 

Abdom Imaging. 2007 May 15; [Epub ahead of print]

 

ACTH-independant macronodular adrenal hyperplasia: imaging findings of a rare condition : A case report.

 

Verma A, Mohan S, Gupta A.

 

Department of Radio Diagnosis, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Rae Bareily Road, Lucknow, 226 014, Uttar Pradesh, India, agupta@sgpgi.ac.in.

 

Endogenous Cushing's syndrome is a relatively rare disease. Most cases being ACTH-dependent, ACTH-independent Cushing's syndrome (AICS) is an even rarer condition [15%-20%]. In more than 95% cases the cause of AICS is unilateral adrenal enlargement caused by adenoma or carcinoma. Bilateral adrenal disease is caused by primary pigmented nodular adrenal dysplasia (PPNAD) and ACTH-independent macro nodular hyperplasia (AIMAH). Only few case reports of the latter condition exist in the radiology literature, PPNAD being the commoner of two as the cause for AICS.

 

Keywords ACTH-independent Cushing?s syndrome (AICS) - ACTH-independent macronodular adrenal hyperplasia (AIMAH) - Adrenal tumor - Bilateral adrenal hyperplasia

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Mary, thanks for this. It is good to know that there is research going on. I wonder if it is like a lot of other HPA axis problems not really so much rare as rarely identified. Nailing the imaging characteristics will help. I wonder how long it usually takes for this kind of info to filter down to the radiologist that read the scans.

 

den

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Absolutely fascinating! I also wonder about the rarity vs. rarely diagnosed realtionship?

If nothing else, this helps some of us to keep our sanity, knowing that the docs are defenitely still discovering the new conditions.

Thanks for sharing this Mary!

LoriJane

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