Testicular adrenal-like tissue in a patient with 17 alpha-hydroxylase deficiency.
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 11 beta-hydroxylase deficiency. We report a case of male pseudohermaphroditism with 17 alpha-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.
García-Mayor
R V
RV
Endocrine Section, General Hospital of Vigo, Galicia, Spain.
Sopeña
B
B
Fluiters
E
E
Páramo
C
C
Fernández Martín
R
R
Galofré
J C
JC
Andrade Olivié
A
A
D000293:Adolescent; D000311:Adrenal Glands / Q000473:pathology*; D000312:Adrenal Hyperplasia, Congenital / Q000150:complications / Q000378:metabolism / Q000473:pathology; D000450:Aldosterone / Q000097:blood; D012734:Disorders of Sex Development / Q000150:complications* / Q000378:metabolism; D006728:Hormones / Q000097:blood; D006801:Humans; D008297:Male; D010641:Phenotype; D013736:Testicular Neoplasms / Q000150:complications* / Q000378:metabolism / Q000473:pathology
D006728:Hormones; D000450:Aldosterone