Success of glucocorticoid replacement therapy on fertility in two adult males with 21-CAH homozygote classic form

Journal of Endocrinological Investigation

Volumn 20, Issue 11, 1997, Pages 690-694

  Valentino, R ^a,b   Savastano, S ^a   Tommaselli, A P ^a   Scarpitta, M T ^a   Dorato, M ^a   Gigante, M ^a   Calvanese, E ^a   Carlino, M ^a   Lombardi, G ^a  

^a UNIVERSITY OF NAPLES FEDERICO II   (Italy)

^b NONE   (Italy)

Author keywords

21 hydroxylase deficiency; Adrenal deficiency; CAH; Fertility potential; Glucocorticoid therapy; Male infertility

Indexed keywords

GLUCOCORTICOID; HYDROCORTISONE;

ADULT; ARTICLE; CASE REPORT; CONGENITAL ADRENAL HYPERPLASIA; GROWTH; HOMOZYGOTE; HORMONE SUBSTITUTION; HUMAN; INTRAMUSCULAR DRUG ADMINISTRATION; MALE; MALE INFERTILITY; MATURATION; ORAL DRUG ADMINISTRATION; SEMEN ANALYSIS; SEXUAL MATURATION; STEROID 21 MONOOXYGENASE DEFICIENCY; TESTIS DEVELOPMENT;

EID: 0031443496     PISSN: 03914097     EISSN: None     Source Type: Journal    
DOI: 10.1007/BF03348034     Document Type: Article

References (20)

1. New M.I., Dupont B., Pang S., Pollack M.S., Levine L.S. An update of congenital adrenal hyperplasia. Rec. Prog. Horm. Res. 37: 105, 1981.
2. Kohn B., Levine L.S., Pollack M.S., Pang S., Lorenzen F., Levy D., Lerner A. J., Rondanini G.F., Dupont B., New M.I. Late-onset steroid 21-hydroxylase deficiency: a variant of classical congenital adrenal hyperplasia. J. Clin. Endocrinol. Metab. 55: 817, 1982.
3. Bongiovanni A.M., Root A.W. The adrenogenital syndrome. N. Engl. J. Med. 268: 1283, 1963.
4. White P.C., New M.I., Dupont B.D. Congenital adrenal hyperplasia. N. Engl. J. Med. 316: 1519, 1987.
5. Pang S., Wallace M.A., Hofman L., Thuline H.C., Dorche C., Lyon I.C.T., Dobbins, R.H., Kling S., Fujieda K., Suwa S. Worldwide experience in newborn screening for classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Pediatrics 81: 866, 1988.
6. Valentino R., Tommaselli A.P., Rossi R., Lombardi G., Varrone S. A pilot study for neonatal screening of congenital adrenal hyperplasia due to 21-hydroxylase and 11β-hydroxylase deficiency in Campania region. J. Endocrinol. Invest. 13: 221, 1990.
7. Fiet J., Pham-Huu-Trung M.T. Radioimmunoassay of 17-hydroxyprogesterone: physiological variations and pathological values in man. J. Steroid Biochem. 10: 569, 1979.
8. Wischusen J., Baker H.W.G., Hudson B. Reversible male infertility due to congenital adrenal hyperplasia. Clin. Endocrinol. 14: 571, 1981.
9. Urban M.D., Peter A.L., Migeon C.J. Adult height and fertility in men with congenital virilizing adrenal hyperplasia. N. Engl. J. Med. 299: 1392, 1978.
10. Korth-Schutz S., Levine L.S., New M.I. Serum androgens in normal prepubertal and pubertal children and in children with precocious adrenarche. J. Clin. Endocrinol. Metab. 42: 117, 1976.
11. Glass A.R., Jackson S.G., Perlstein R.S., Wray H.L. Adrenal insufficiency in a man with non-classical 21-hydroxylase deficiency: consequence or coincidence? J. Endocrinol. Invest. 17: 665, 1994.
12. Azziz R., Zacur H.A. 21-hydroxylase deficiency in female hyperandrogenism: screening and diagnosis. J. Clin. Endocrinol. Metab. 69: 577, 1989.
13. Ehrmann D.A., Barnes R.B., Rosenfield R.L. Polycystic ovary syndrome as a form of functional ovarian hyperandrogenism due to dysregulation of androgen secretion. Endocr. Rev. 16: 322, 1995.
14. New M.I., Gertner J.M., Speiser P.W., del Balzo P. Growth and final height in classical and non-classical 21-hydroxylase deficiency. Acta Pediatr. Jpn. (Suppl.) 30: 79, 1988.
15. Zerah M., Ueshiba H., Wood E., Speiser P.W., Crawford C., McDonald T., Pareira J., Gruen D., New M.I. Prevalence of non classical steroid 21-hydroxylase deficiency based on a morning salivary 17-hydroxyprogesterone screening test: a small sample study. J. Clin. Endocrinol. Metab. 70: 1662, 1990.
16. Pang S., Wallace M.A., Hofman L., Thuline H.C., Dorche C., Lyon I.C.T., Dobbins, R.H., Kling S., Fujieda K., Suwa S. Worldwide experience in newborn screening for congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Pediatrics 81: 866, 1988.
17. Miller W.L. Genetics, diagnosis, and management of 21-hydroxylase deficiency. J. Clin. Endocrinol. Metab. 78: 241, 1994.
18. Mellon S.H., Miller W.L. Extra-adrenal steroid 21-hydroxylation is not mediated by P450c21. J. Clin. Invest. 84: 1497, 1989.
19. 5-steroids in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. J. Clin. Endocrinol. Metab. 78: 299, 1994.
20. Prader A., Zachmann M., Illig R. Fertility in adult males with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Acta Endocrinol. (Copenh.) (Suppl.) 177: 57, 1973.