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-  2017 

NONCLASSICAL 21-HYDROXYLASE DEFICIENCY PRESENTED AS ADDISON’S DISEASE AND BILATERAL ADRENAL INCIDENTALOMAS

DOI: 10.4183/aeb.2017.232

Keywords: Nonclassical 21-hydroxylase deficiency, congenital adrenal hyperplasia (CAH), Addison’s disease, adrenal incidentaloma, myelolipoma, hypoadrenocorticism

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Abstract:

21-hydroxylase deficiency (21 OHD) is the most common form of congenital adrenal hyperplasia (CAH) and it has been widely described in the literature. Adrenocortical incidentalomas are unfrequently the presenting manifestations of CAH, especially in nonclassical form of 21 OHD (NC 21 OHD). Myelolipoma has previously been reported more frequently than other adrenal adenomas associated with CAH

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