Abstract
In untreated congenital adrenal hyperplasia due to 21-hydroxylase deficiency (21OHDS) the presence of adrenal and testicular tumors had been described; however little is known about the effect of the enzymatic defect on fertility in males. We studied a male adult patient affected by 21OHDS for infertility, after a long period of discontinuation of glucocorticoid therapy and then during resumption of treatment and 8 months after monoadrenalectomy. The initial spermatic count revealed azoospermia and testicular needle aspiration showed a cytological picture consistent with prepuberty. The morphofunctional study revealed a right adrenal mass with reduced uptake at radioscan. Treatment was resumed with onset of impotency, which improved after reduction of the dose of glucocorticoids. The patient was monoadrenalectomised and his spermatic count increased. The patient shows that corticosteroid therapy in 21OHDS should be continued lifelong to avoid adrenal hyperplasia with possible areas of autonomy and to allow regular fertility. Impotence during treatment is probably due to a decrease of excessive adrenal androgens while testicular androgen production is still suppressed.
| Original language | English |
|---|---|
| Pages (from-to) | 41-43 |
| Number of pages | 3 |
| Journal | Experimental and Clinical Endocrinology and Diabetes |
| Volume | 111 |
| Issue number | 1 |
| DOIs | |
| Publication status | Published - Feb 2003 |
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Keywords
- 21-Hydroxylase deficiency syndrome
- Adrenal mass
- Impotence
- Infertility
ASJC Scopus subject areas
- Endocrinology
- Endocrinology, Diabetes and Metabolism
Cite this
Unilateral adrenal tumor, erectile dysfunction and infertility in a patient with 21-hydroxylase deficiency : Effects of glucocorticoid treatment and surgery. / Scaroni, Carla; Favia, G.; Lumachi, F.; Opocher, G.; Bonanni, G.; Mantero, F.; Armanini, D.
In: Experimental and Clinical Endocrinology and Diabetes, Vol. 111, No. 1, 02.2003, p. 41-43.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Unilateral adrenal tumor, erectile dysfunction and infertility in a patient with 21-hydroxylase deficiency
T2 - Effects of glucocorticoid treatment and surgery
AU - Scaroni, Carla
AU - Favia, G.
AU - Lumachi, F.
AU - Opocher, G.
AU - Bonanni, G.
AU - Mantero, F.
AU - Armanini, D.
PY - 2003/2
Y1 - 2003/2
N2 - In untreated congenital adrenal hyperplasia due to 21-hydroxylase deficiency (21OHDS) the presence of adrenal and testicular tumors had been described; however little is known about the effect of the enzymatic defect on fertility in males. We studied a male adult patient affected by 21OHDS for infertility, after a long period of discontinuation of glucocorticoid therapy and then during resumption of treatment and 8 months after monoadrenalectomy. The initial spermatic count revealed azoospermia and testicular needle aspiration showed a cytological picture consistent with prepuberty. The morphofunctional study revealed a right adrenal mass with reduced uptake at radioscan. Treatment was resumed with onset of impotency, which improved after reduction of the dose of glucocorticoids. The patient was monoadrenalectomised and his spermatic count increased. The patient shows that corticosteroid therapy in 21OHDS should be continued lifelong to avoid adrenal hyperplasia with possible areas of autonomy and to allow regular fertility. Impotence during treatment is probably due to a decrease of excessive adrenal androgens while testicular androgen production is still suppressed.
AB - In untreated congenital adrenal hyperplasia due to 21-hydroxylase deficiency (21OHDS) the presence of adrenal and testicular tumors had been described; however little is known about the effect of the enzymatic defect on fertility in males. We studied a male adult patient affected by 21OHDS for infertility, after a long period of discontinuation of glucocorticoid therapy and then during resumption of treatment and 8 months after monoadrenalectomy. The initial spermatic count revealed azoospermia and testicular needle aspiration showed a cytological picture consistent with prepuberty. The morphofunctional study revealed a right adrenal mass with reduced uptake at radioscan. Treatment was resumed with onset of impotency, which improved after reduction of the dose of glucocorticoids. The patient was monoadrenalectomised and his spermatic count increased. The patient shows that corticosteroid therapy in 21OHDS should be continued lifelong to avoid adrenal hyperplasia with possible areas of autonomy and to allow regular fertility. Impotence during treatment is probably due to a decrease of excessive adrenal androgens while testicular androgen production is still suppressed.
KW - 21-Hydroxylase deficiency syndrome
KW - Adrenal mass
KW - Impotence
KW - Infertility
UR - http://www.scopus.com/inward/record.url?scp=0037296204&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0037296204&partnerID=8YFLogxK
U2 - 10.1055/s-2003-37499
DO - 10.1055/s-2003-37499
M3 - Article
VL - 111
SP - 41
EP - 43
JO - Experimental and Clinical Endocrinology and Diabetes
JF - Experimental and Clinical Endocrinology and Diabetes
SN - 0947-7349
IS - 1
ER -