Testicular Adrenal-Like Tissue in a Patient with 17α-Hydroxylase Deficiency

Abstract
Testicular adrenal-like tissue (TALT) have been observed in patients with congenital adrenal hyperplasia, and is usually associated with 21-hydroxylase deficiency; in 3 cases with 11β-hydroxylase deficiency. We report a case of male pseudohermaphroditism with 17α-hydroxylase deficiency (17OHD) who also had TALT. To our knowledge, this is the first report about the association of 17OHD and TALT. Also, the patient had high levels of serum aldosterone – an unusual finding in patients with 17OHD. A possible pathogenic mechanism is discussed.

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